Chapter 21 345

Musculoskeletal System

Chris M. Senter · Deborah Tomlinson

Contents The two predominant primary bone tumors in chil- 21.1 Limb Salvage Procedures ...... 345 dren are osteosarcoma (60%) and Ewing’s sarcoma 21.1.1 Incidence ...... 345 21.1.2 Procedure ...... 345 (34%). (Solid tumors are detailed in Chapter 2.) 21.1.2.1 Management ...... 348 Combined, these are the most predominant causes of 21.2 Amputation ...... 348 bone tumors in the lower extremities (Nagarajan et 21.2.1 Incidence ...... 348 al., 2002). Upper- and lower-extremity bone tumors 21.2.2 Procedure ...... 349 are effectively treated with multimodal treatment 21.2.3 Rotationplasty ...... 349 21.2.3.1 Management ...... 349 that often involves local control by one of two surgi- 21.2.3.2 Comparison of Limb Salvage cal options: and Amputation ...... 350 ▬ Duration of Survival ...... 350 Limb-sparing resection Immediate and Ultimate Morbidity ...... 350 ▬ Amputation Function ...... 350 Quality of Life ...... 351 21.3 Altered Bone Density and Increased Risk of Fracture ...... 351 21.1 Limb Salvage Procedures 21.3.1 Incidence ...... 351 21.3.2 Etiology ...... 351 21.1.1 Incidence 21.3.3 Treatment ...... 352 21.3.4 Prognosis ...... 352 Limb-salvage procedures are feasible in approxi- References ...... 352 mately 80% of childhood sarcomas (Kumta et al., 2002).

21.1.2 Procedure The decision to proceed with limb-salvage surgery must consider the following: ▬ Aggressiveness of the underlying tumor and its stage ▬ Need to achieve a satisfactorily wide excision of the tumor ▬ Ability of the reconstructed extremity to be at least as functional as an ablative procedure and prosthesis ▬ Response to neo-adjuvant therapy (Kumta et al., 2002; Nagarajan et al., 2002) 346 Chapter 21 C. M. Senter · D.Tomlinson Fracture inter- by treated nal fixation Loosening children with large children with large potentialgrowth union Delayed or nonunion major musculoten- dinous units prosthesis to remaining femur resection by the defect created tibial articularproximal surface use of extremity Bone resorption. donors (removed with ligaments,donors (removed sizing, for x-rayed and frozen). use,Before allograft is cultured, involvement.placed in antibiotic solution,and thawed. bone Autogenous also be used.(hip or fibula) may segment Large defects usually require over Grafted bone is incorporated complications. bonetime with native survival is good allografts over autogenous grafts (60–80%) despite or casting Bracing periods of non- prolonged time weightbearing. be stable for Can Infection – may ment with metallic implant used bone absence of achieve to replace bone and joint.to be necessary may osseous healing components:Implant has four allograft and – of removal require Metallic stem that anchors period healing. Stable joint. High incidence of weightbearing. antibiotics. Immediate time decreases over use of prolonged Rapid functional i.e., of loosening complications – usually Fracture bone (tibia or femur) stem within the – that fills Metallic distal femur – replacement of Prosthetic – Rotating hinge knee joint bonenative attachment of for and adjacent joint. Joint is then using plating and autogenous fixated bone or allografts and ankle) (occasionally wrist and functional reconstruction for inability to bend knee. (particularly with allograft adult a young Not sufficient for bone) Types of limb-sparing reconstruction and amputation (Nagarajan et al.,Types 2002) Allograft bone Allograft bones from deceased without joint Any Overall allograft need protracted May Nonunion – Endoprosthetic Resection of entire segment implants tumor and replace- bone involving Distal femur Postoperative Survival of prosthesis Infection rehabilitation. progressively loosening, Aseptic Composite Composite endoprosthetic allograft to bone that serves as site allografts of articulating Attachment prosthesis femur Proximal tibia Proximal of attachment to remaining Stable joint, incorpo- of additional ration periods Protracted of non-weight- humeral Proximal endo- – As for bone, and allows prostheses and bearing allografts Type of Type reconstruction of procedureArthrodesis Brief description resection of the tumor Involved Knee, shoulder Joint or bone affected commonly Extremely stable limitations – Physical Infection Advantages Disadvantages Complications Table 21.1. Musculoskeletal System Chapter 21 347 pain Infection ntains a small internal sm within the prosthesis a of supporting struc- limb tures of affected – Aseptic loosening – Infections while providing child. ambulation for Replace when adult height has been reached without complete amputation main types:Four – (below-the-knee) Transtibial – Knee disarticulation due to high femoral tumors when tion for tumors in distal femur therapythe back of thigh, neuro- leaving possible bundle and the distal end vascular of tibia and foot.The of the distal femur leg is rotated incidence of bone 180 degrees and tibia is reattached (Van Nes rotationplasty) with blood vessels the femur to struction, useful nally, stable recon- problems is not limb salvage reconnected.The ankle is now at the issues psychosocial of the knee,level that and the foot the limb following greater Possible reconstruction limb Phantom is now pointing backwards acts as the wound Delayed with an stump which can be fitted Stump pain artificial of a below- limb at the level healing knee amputation knee movement, con- less energy fewer Potentially is less well sumption. accepted No phantom surgeries. limb pain (Kotz, 1997) Disturbances in circulation thromboses) (particularly – amputation (above-knee) Transfemoral – Hip disarticulation Bone overgrowth several interventionsseveral from turning of a key within implant to replacement or of spacers that increase in size number as the child grows 10 in girls). of 12 in boys, and Lengthens revision – Prosthesis – Replacement for growth provides prosthesis failure prosthesis – Larger (Continued) Amputation of tumor is impossible Wide excision Majority are trans- wide resec- Provides chemo-Rotationplasty delay May from and soft tissues removed Tumor Stump-prostheses Majority tumors for functio- Impressive Appearance of Infection magnet, source.This an external by power is activated limb to be placed in a box, enables the affected a mechani which stimulates 1 extendto approximately mm every 20 minutes, further without the need for intervention surgical An extendable prosthesis is now beginning to be replaced by a noninvasive lengthening device.TheAn extendable a noninvasive prosthesis is now beginning to be replaced by extendable prosthesis,which co Type of Type reconstruction of procedureExpanding Brief description endoprosthetic knee joint. Implant has a mechanism implants Implants inserted as endoprosthetic extremities Lower growth”achieved“active by for children with For Joint or bone affected commonly Multiple surgeries loosening Aseptic potential growth Advantages be required may Disadvantages Infection >4–5 cm (bone age – Limb lengthening Complications Table 21.1. a 348 Chapter 21 C. M. Senter · D.Tomlinson

Various options are available for skeletal recon- Table 21.2. Limb-sparing postoperative nursing care guide- struction, and the type used usually depends on the lines for dressing changes (from Gilger et al., 2002) following: Dressing changes 1. Site of the tumor First dressing change is done by surgical staff 5–7 days 2. Patient’s age following surgery 3. Patient’s prognosis After this time, dressing is changed about every 3 days 4. Orthopedic surgeon’s expertise Frequency of dressing changes is very dependent Table 21.1 provides a general outline of the types of on healing limb-sparing reconstructions that are available. However, bones such as ribs, clavicles, digits, scapu- lae, and fibulae can be removed with adequate mar- Postoperative management regarding dressing gins and with no effect on function. changes and rehabilitation of a patient following limb-sparing surgery is briefly highlighted in 21.1.2.1 Management Tables 21.2 and 21.3, respectively. Rehabilitation can take 2 years, by which time the patient should have a Management is complicated for these patients who functioning limb. face life-threatening illness exacerbated by life- changing surgery. Heightened awareness should be made to psychosocial care. Nursing care encompass- 21.2 Amputation es meeting important physical and psychosocial 21.2.1 Incidence needs.Patients receiving limb-sparing surgery of this nature will often be adolescents, and any change in Before the 1980s, amputation was the main surgical body image may be detrimental to their psychologi- option for malignant bone tumors. Currently per- cal well-being. The nursing challenge is to help ado- formed in less than 15% of patients,amputation is re- lescents balance the limits of their illness and recov- served for patients with widespread local disease ery with normal developmental needs (Dealy et al., where it is necessary to achieve wide excision.Howev- 1995). er, the procedure includes the premise that the result- ant limb reconstruction be as functional as possible.

Table 21.3. Guidelines for limb-sparing postoperative rehabilitation (from Gilger et al., 2002)

Recovery Day 1 Day 2 Day 3 Outpatient follow-up

Initiate use of continuous Encourage sitting in bed Continue CPM Ambulate Check patient notes passive motion (CPM). as much as possible to when in bed with crutches and protocol for informa- Early mobility decrease chances of ortho- tion regarding use of – Decreases risk of static hypotension during brace, assistance with contractures transfers. ambulation, use of CPM, – Facilitates early Physiotherapy referral etc. ambulation Encourage open discussion – May decrease pain regarding pain. For transfer and while sitting, use orthosis to maintain extension to avoid knee flexion contractures Musculoskeletal System Chapter 21 349

21.2.2 Procedure Table 21.4. Amputation postoperative nursing care guide- lines for dressing changes Amputation in children raises several issues because Dressing changes of the following important considerations: Casts generally applied during surgery ▬ The etiology of childhood limb deficiencies ▬ Expected skeletal growth First cast change within 10 days after surgery ▬ Functional demand on the locomotor system and Number of changes depends on extent of edema, prosthesis infection, and muscle involvement ▬ Appositional bone stump overgrowth ▬ Psychological challenges (Krajbich, 1998) occurs, the stump becomes shorter, function be- Despite the increased use of limb-sparing proce- comes limited, and further surgery may be necessary. dures, amputation remains a valuable procedure. A It is also recognized that in children under the age of tumor that extends into an adjacent joint involving 12 years, further bone growth may occur at the ends blood vessels and nerves would be impossible to re- of long bones, which would also necessitate further move without contaminating the surgical margins, surgical intervention. increasing the risk of local recurrence. The limb’s function and movement are important, and inade- 21.2.3 Rotationplasty quate surgical intervention may risk long-term sur- vival.Amputation and an artificial limb may offer the Rotationplasty is a surgical procedure that is consid- best surgical option and function (see Table 21.1). ered a method of amputation because it involves to- It is important to decide the level of local bone re- tal removal of the tumor and surrounding tissue section to ensure adequate tumor clearance and suf- without reconstruction. A brief outline of the proce- ficient tissue availability to ensure skin closure. A dure is included in Table 21.1. This surgical option is measurement of 13 cm above the radiological visible always discussed in relation to treatment because of tumor has been reported as the appropriate level; its functional outcomes.However,in the United King- however, a more distal level of amputation offers the dom, it is a choice rarely made by patients and par- best functional level. ents. In the lower extremities, above-knee amputation is the most common level of amputation due to the 21.2.3.1 Management frequent presentation around the knee joint, and be- low-knee amputation is done for tumors of the distal Postoperative management regarding dressing tibia. In the upper limb, tumors tend to present more changes and rehabilitation of a patient following am- commonly at the proximal humerus.If limb salvage is putation surgery is briefly highlighted in Tables 21.4 not possible due to the extent of disease, then disar- and 21.5, respectively. Phantom limb pain can be a ticulation and forequarter amputation of the shoul- particular problem (described in Chapter 28), and der maybe required. This level is very disabling, and appropriate aggressive pain management is required artificial limbs are often for cosmetic purposes only. immediately post amputation in an attempt to avoid In children, if the aim is to restore function to the chronic phantom limb pain. limb, it is crucial to consider how much potential When limb preservation is not possible, patients skeletal growth they still have before undertaking often view amputation as failure and begin grieving surgery. The distal epiphyseal plates provide 70% of not only the potential loss of the limb but also the be- the potential longitudinal growth of the femur (Na- lief that life will never be normal again. Restoring in- garajan et al,.2002).Initially,if this plate is lost in sur- dividual belief in acquiring some form of normal ac- gery, the amputated limb is functional. But as growth tivity with realistic goals is paramount. Depending 350 Chapter 21 C. M. Senter · D.Tomlinson

Table 21.5. Guidelines for amputation postoperative rehabilitation (from Gilger et al., 2002)

Day 1 Day 2 Day 3 Outpatient follow-up

Encourage sitting in bed as much as possible Ambulate Check patient notes and to decrease chances of orthostatic on crutches protocol for information hypotension during transfers. regarding use of brace, Physiotherapy referral assistance with ambulation, Encourage discussion about phantom use of CPM, etc. (neuropathic) pain/sensation. Do not remove cast – provides protection and controls edema. Do not elevate limb after first 24 hours unless instructed by physician – prolonged hip flexion can lead to contractures

on the child’s age, preamputation assessment and 21.2.3.2 Comparison of Limb Salvage preparation are offered. The involvement of a play and Amputation specialist can be beneficial to help prepare children and explain what will happen. The opportunity to Many studies have investigated the comparisons be- visit a local artificial limb center prior to admission tween limb salvage and amputation with regard to has been shown to be not only beneficial preopera- ▬ Duration of survival tively but appears to help the individual cope better ▬ Immediate and ultimate morbidity following surgery. Meeting someone who has had an ▬ Function amputation at the same level can be inspiring and ▬ Quality of life create a role model as to what can be achieved. For children who are admitted for amputation, the Duration of Survival stay in hospital is quite short. Once over the initial surgery and with adequate pain management, dis- Survival rates and local recurrence rates have been charge follows soon after the individual is able to comparable in both groups (Sluga, 1999; Weiss, 1999; transfer and mobilize safely. Function, movement, Bacci et al., 2003). balance, and safety are part of the physiotherapist’s rehabilitation program for working on daily activi- Immediate and Ultimate Morbidity ties. The occupational therapist helps promote inde- Complications following limb-sparing procedures pendence by providing equipment; advice, and refer- occur more frequently than in amputation proce- rals to local services to help patients and their fami- dures (Nagarajan et al., 2002). These complications lies achieve realistic goals.Ideally seen in hospital ini- can directly impact future function and quality of tially by the limb rehabilitation team, the patient will life. be referred back to his or her local artificial limb cen- ter for prosthetic rehabilitation. Function Younger children may lack or have only limited Functional outcome is complex and based on more understanding and will need to be constantly moni- than physical ability (Nagarajan et al., 2002). A com- tored to reinforce the need to exercise to maintain monly used assessment of function is the Muscu- movement. If exercises are not maintained, then loskeletal Tumor Society scoring system. This is a more problems will develop. physician assessment based on the patient’s overall pain, level of activity restriction, emotional accept- ance, use of supports, walking, and gait. Another as- Musculoskeletal System Chapter 21 351 sessment is the self-administered questionnaire of the Toronto Extremity Salvage Score (TESS), which 21.3 Altered Bone Density divides functional outcome into and Increased Risk of Fracture 1. Disability, referring to inability or restriction in 21.3.1 Incidence performing normal activities Children with malignancy are at risk of developing 2. Handicap, referring to the inability to assume, or osteopenia.Van der Sluis et al. (2002) have reported a limitation in assuming, a role that is normal for fracture risk six times higher in children with acute that person (depending on age, gender, social, and lymphoblastic leukemia (ALL) compared with cultural factors) (Davis et al., 1996). healthy controls. The risk of fractures increases con- The difficulty in determining whether functional dif- siderably during treatment and for a period after ferences are notable is the lack of consistency in treatment. measurements of function.Generally it would appear that any functional differences are small (Nagarajan 21.3.2 Etiology et al., 2003; Davis et al., 1999), but reports have also indicated significant improved function with limb- In childhood ALL,skeletal changes are often detected saving surgery when compared with ablative surgery at diagnosis, including osteolysis, sclerosis, osteo- (Renard et al., 2000). More consistently, however, re- porosis, and, occasionally, pathological fractures ports state that functional outcome following rota- (Arikoski et al., 1999). These changes are probably tionplasty is superior to that of amputation (Heeg caused by the disease process and alterations in min- and Torode, 1998; Hillman et al., 2000; Fuchs et al., eral homeostasis and bone mass. 2003). In addition,methotrexate,radiotherapy,and corti- costeroids are some of the adjuvant antineoplastic Quality of Life treatments currently used and have been described Definitions of quality of life have varied; therefore, as hindering normal development of bone mass comparison between studies on quality of life has (Nysom et al., 2001). (Some molecular mechanisms been difficult, and reports on differences in quality of are outlined in Table 21.6.) In the older age group, the life between these patients have varied in their con- bone mass is calculated on the degree of bone loss clusions. Certainly, it would appear that there is little and the maximum bone mass acquired in the 2nd difference (Refaat et al., 2002; Lane et al., 2001; Post- and 3rd decades of life. If the normal acquisition of ma, 1992), despite predictions that the quality of life bone mass is disrupted during childhood and adoles- of patients undergoing limb-salvage procedures cence,survivors of childhood cancer may develop os- would be superior to that of patients undergoing am- teopenia, osteoporosis, and pathological fractures in putation. There are little data available regarding later life (Azcona et al., 2003). Chemotherapy drugs quality of life measurements in children following such as ifosfamide and cisplatinum used in the treat- limb-salvage or amputation procedures. ment of bone tumors may interfere with the process Despite the higher complication rate and equivo- of renal calcium and vitamin D metabolism. During cal improved quality of life, limb-sparing surgery re- treatment for ALL, persistent low levels of bone alka- mains current practice at most centers (Nagarajan et line phosphatase have indicated a potential flaw in al., 2002). This may be due to a perception that “sav- osteoblast differentiation.Azcona et al.(2003) further ing” the limb is of utmost importance. suggest that many other factors may affect bone min- The management of these patients includes an in- eralization in children with malignancy, including dividualized surgical plan and an awareness of their long periods of bed rest, poor diet, growth hormone psychosocial needs after surgery. deficiency (see Chapter 23),and variations in vitamin D metabolism. Diagnoses of these anomalies are of- ten made by radiological imaging. However, it is im- 352 Chapter 21 C. M. Senter · D.Tomlinson

Table 21.6. Molecular mechanisms accounting for loss of bone mass (Cohen, 2003)

Treatment Mechanism in bone loss

Methotrexate Appears to increase bone resorption and excretion Glucocorticoids Prevent 1-alpha-hydroxylation of vitamin D in the kidneys to form the active metabolite 1,25-dihydroxy-vitamin D; this leads to impaired intestinal absorption of calcium. Inhibit the expression of vitamin D receptor in bone. Inhibit production of osteocalcin (principle bone matrix protein). Decrease local production of cytokines, which inhibit bone resorption Bone marrow transplant Increase in bone marrow interleukin-6, which may stimulate bone resorption Differentiation of bone marrow stromal cells into osteoblasts is impaired

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