Documente Academic
Documente Profesional
Documente Cultură
Contents Abstract
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4252 In recent years, the treatment for children
with bone tumours has advanced. These
Aetiology and Classification . . . . . . . . . . . . . . . . . . . . . . 4252
advances are largely due to chemotherapeutic
Anatomy, Pathology and Physiology . . . . . . . . . . . . 4255 developments, although the basis of good
Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4255 treatment remains highly specialized, experi-
Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4255 enced and multi-disciplinary teamwork. The
Imaging Methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4255 corresponding increase in survival combined
Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4256
with the demanding functional requirements
Non-Surgical Treatment of Bone Tumours . . . . . 4257 of these young patients challenge the surgeon
Ewings Sarcoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4257
with limb salvage and reconstruction proce-
Osteosarcoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4257
dures. Surgery is carried out in two steps.
Indications for Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . 4258 Firstly, en bloc resection and secondly recon-
Pre-Operative Preparation and Planning . . . . . . 4258 struction. The range of possibilities involving
Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4259 the available biological and non-biological
Resection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4260 materials is broad. The success of such surgery
Reconstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4262 in conserving a limb hinges on thoughtful and
Post-Operative Care and Rehabilitation . . . . . . . . 4271 creative pre-operative design and planning.
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4273
Peri-Operative Complications . . . . . . . . . . . . . . . . . . . . . 4273 Keywords
Post-Operative Complications . . . . . . . . . . . . . . . . . . . . . 4275 Aetiology and classification Chemotherapy
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4278 Children Complications Diagnosis
Ewings sarcoma Limb salvage Non-
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4278
surgical treatment Osteosarcoma Recon-
struction-allografts, extending prosthesis,
bone lengthening Resection, growth-plate
preservation, joint preservation, physeal dis-
traction, intra-epiphyseal osteotomy Reha-
bilitation Surgical indications Surgical
M. San-Julian (*)
Techniques
Department of Orthopaedic Surgery, University of
Navarra, Pamplona, Spain
e-mail: msjulian@unav.es
B.L. Vazquez-Garca L. Sierrasesumaga
University of Navarra, Pamplona, Spain
A small percentage of osteosarcomas are central Currently, most research into sarcomas
or superficial tumours of low grade (low-grade focusses on the altered genetic pathways,
central osteosarcoma, parosteal osteosarcoma, the main objectives being to identify prognostic
periosteal osteosarcoma). However, most osteosar- factors and to find the causes for resistance
comas are high-grade and intramedullary; they are to chemotherapy. We trust these studies will,
classified by their predominant histological in the future, find new therapies that are
subtype (osteoblastic, chondroblastic, fibroblastic, more effective in the treatment of sarcomas:
and telangiectatic). There also exists an extra- despite great advances, 30 % of affected children
skeletal osteosarcoma, but this is a rarity. still do not survive the disease.
Fig. 1 (continued)
4254 M. San-Julian et al.
Fig. 1 (a) Ewings sarcoma in a 9-month-old baby. The from the growth-plate. (c) The tumour was resected en-
tumour is in contact with the most important growth-plate bloc and reconstruction was carried out with grafts of
of the skeleton. (b) Consecutive X-ray sequence of autologous bone from the ipsilateral fibula and tibia. (d)
response to neo-adjuvant chemotherapy. The tumour Incorporation of the graft was excellent, as was function.
became progressively smaller, moving away from the Six years later, the patient remains free of disease. No
physis. The cortex enlarged, and healthy new bone arose further limb length discrepancy was observed
Limb Salvage in Children 4255
Proliferative Zone
Epiphysis
CT. This is a useful method for seeing Bone Scintigraphy. This is used to rule out
fractures and diagnosing tumours in areas of multifocal lesions, skip metastasis etc.
complex anatomy, such as the scapula, pelvis Angiography. This technique provides infor-
and sacrum. Contrast is used for studies mation useful in the evaluation of the vascu-
involving large neurovascular structures or larization of a neoplasm, which is important
highly vascularized lesions. when planning surgery. In some cases, angi-
Magnetic resonance imaging. MRI is ography is used as a therapeutic procedure: to
a fundamental tool in the study of the intra- administer intra-arterial chemotherapy or to
and extra- osseous extension of tumours, in perform an embolization of hypervascularized
determination of the involvement of soft tis- zones.
sues and neurovascular structures, and in the In summary, diagnosis is made on the basis of
evaluation of peritumoural oedema. It is not as basic X-ray techniques; tumour extension is
satisfactory as CT for evaluating destruction determined by magnetic resonance.
of the cortex, microfractures and calcification
in the tumour matrix. T1-weighted sequences
increase the contrast between tumour and Pathology
bone, bone marrow and adipose tissue. T2-
weighted sequences increase the contrast The biopsy of an osseous lesion can have signifi-
between tumour and muscle; in addition, T2 cant prognostic and therapeutic consequences.
sequences accentuate peritumoural oedema. A badly-done biopsy can seriously jeopardize the
Gadolinium contrast is useful to study vascu- possibilities of conservation of the affected limb or
larization of a tumour and to evaluate even compromise the prognosis. Therefore, biopsy
response. should be done by the team that is going to carry
Limb Salvage in Children 4257
out the definitive treatment of the patient. Recent Although retrospective series from individual
research at various centres has shown that institutions indicate that local control and
a quarter of surgical biopsies of musculoskeletal survival are better with surgery rather than
neoplasms are carried out in an inappropriate way with radiotherapy, the majority of these studies
[15, 16, 18], incorrectly interpreted or both. are compromised by a bias in the selection of
For an experienced pathologist practiced in patients. For patients subjected to macroscopic
this area, a needle biopsy or Tru-cut biopsy will resection but with residual microscopic disease,
be sufficient. Open biopsy can be indicated when the value of adjuvant radiotherapy is disputed
the sample obtained by needle is not representa- [23]. It is not known whether higher
tive enough. Immunohistochemistry can be doses of radiotherapy might improve results. In
a useful complement to conventional histology. general, surgery is used as the definitive
With some tumours, such as Ewings sarcoma, in local treatment when a patient is suitable;
which an 1122 chromosomal translocation is radiotherapy is reserved for patients with
characteristic, diagnosis can be established easily unresectable disease or for whom surgery would
by means of PCR techniques. Searching for DNA suppose serious morbidity. Adjuvant radiot-
transcripts in peripheral blood can be useful in the herapy must be considered for patients with resid-
follow-up of patients. ual microscopic disease and/or inadequate
surgical margins that leave residual disease in
the tumour bed.
least 90 % necrosis of the primary tumour after receive post-operative chemotherapy with the
induction chemotherapy have a better prognosis same drugs as administered pre-operatively.
than patients presenting less necrosis [27]. These patients are randomly assigned additional
Patients with less than 90 % necrosis in the pri- treatment with the pegylated form of interferon
mary tumour after initial chemotherapy have alpha 2b. Patients with a standard histological
a higher rate of relapse in the first 2 years in response (10100 % of the tumour is viable)
comparison to patients with at least 90 % necrosis also receive post-operative chemotherapy with
[27, 28]. Imaging techniques, such as dynamic the same drugs as administered pre-operatively,
magnetic resonance imaging and positron emis- but are randomly assigned additional treatment
sion tomography (PET), are currently being with more or less cycles of ifosfamide and
investigated as possible non-invasive methods etoposide.
of evaluating response. Less necrosis must not
be interpreted as meaning that the chemotherapy
has been ineffective: cure rates for patients with Indications for Surgery
little or no necrosis after induction chemotherapy
are much higher than those rates in patients who Most patients should be considered for surgery
do not receive such chemotherapy. (even if metastases are present). The different
In some studies, the degree of tumour necrosis types of limb salvage surgery can be classified
is used to determine the post-operative chemo- by location of the tumour:
therapy. In general, if the tumour necrosis Joint resections.
exceeds 90 %, the pre-operative chemotherapy Diaphysis resections:
regime is continued. If the necrosis is less than Epiphysiolysis prior to resection.
90 %, some groups have added drugs not previ- Intra-epiphyseal or metaphyseal osteotomy.
ously used in the pre-operative treatment. This According to the implanted material, recon-
approach is based on the first reports from the structions can be:
Memorial Sloan-Kettering Cancer Center Biological: allografts or autografts.
(MMSKC) that indicated that results in patients Non-biological: megaprosthesis or expand-
with less than 90 % tumour necrosis were able prosthesis.
improved by adding cisplatin to post-operative Combinations of prosthesis and allograft.
chemotherapy. With a longer follow-up, how-
ever, the results in patients with less than 90 %
tumour necrosis and treated in the MSKCC were Pre-Operative Preparation
the same whether patients received cisplatin in and Planning
the post-operative stage of treatment or not.
Subsequent studies carried out by other groups The best moment for surgery must be agreed by
have not demonstrated an increase in survival consensus with the rest of the team treating the
when drugs not included in the pre-operative patient, so as not to interfere with the chemother-
regimen were added to the post-operative apy protocol. In general, patients receive several
treatment [27, 29]. cycles of neo-adjuvant chemotherapy before
The Childrens Oncology Group (COG), with undergoing surgery because the chemotherapy
the collaboration of several European groups, has facilitates limb-conservation surgery. Chemo-
started a study in which all patients receive a pre- therapy can delay surgery; and surgery, if
operative treatment with doxorubicin, cisplatin, complications (for example, infection) are
and high-dose methotrexate. After surgery, encountered, can delay administration of the che-
patients are divided into two groups on the basis motherapy adjuvant.
of histological degree of necrosis in the resected When designing limb salvage surgery, our
primary tumour. Patients with favourable histol- basic tool is magnetic resonance imaging. In the
ogy (less than 10 % of the tumour is viable) all first consultation, the patient has a baseline
Limb Salvage in Children 4259
MR image, and before surgery a second MR in order to determine the anatomy of the
image is used to evaluate the response to neo- nerve-vessel bundle, the length of the tumour,
adjuvant chemotherapy. The information from the mass of soft tissues, etc. In addition, prepara-
the image is used to design our approach to resec- tions must be made with regard to the material to
tion. The incision must allow us to deal with the be used in reconstruction.
principle blood vessels and nerves. Current com-
puter software allows us to measure the length of
the bone that we are going to resect and also Surgical Technique
assess the degree of soft tissue involvement.
Once the size of the tumour is known, the Conservative surgery for malignant bone
approach has been planned and the anatomy of tumours has two phases: resection and
the nerve-vessel bundle determined (Fig. 3), we reconstruction.
must turn our thoughts to the material for recon- To be able to cure a malignant bone tumour, it
struction. If a bone bank is available, the most is usually necessary to resect en-bloc, with safety
appropriate bone allograft must be selected. margins, the whole diseased area together with
The bone should preferably be from a young the biopsy course [30]. If a tourniquet is used an
male, because the greater extra-medullary diam- Esmarch bandage must not be used. The
eter is useful for establishing good internal fixing. approach route should be chosen on the basis of
The bone should be of appropriate length and tumour location, possible involvement of
from the same side of the body as that which is nerve-vessel structures (usually they are found
it going to substitute. In the case of placing to be displaced but not infiltrated), the location
a megaprosthesis or expandable prosthesis, the of the biopsy taken previously, the technique
same considerations apply. Many such prostheses for reconstruction to be used, etc. The tumour
are custom made and should be ordered from the should be completely removed as a whole,
relevant company, stating size and side, during without being seen. A good safety margin
the period of neoadjuvant chemotherapy. is not a question of centimeters, but rather
In summary: surgery cannot be approached of resecting the tumour through healthy tissue:
without first having studied a current MR image it is not a question of quantity but quality.
4260 M. San-Julian et al.
Rate of local recurrence is a good indicator with Physeal distraction has been extensively used
which to evaluate the quality of resection and the for bone lengthening and for correcting
efficiency of neo-adjuvant chemotherapy treat- angular deformities. Canadell described its use
ment. The planned reconstruction must never be in facilitating the excision of malignant hone
put before quality of resection: a nice post-operative tumours of the metaphysis while preserving the
X-ray is useless if the patient does not survive epiphysis.
or suffers local recurrence of his or her illness. The indications for this technique are:
1. Location of the tumour in the metaphyseal
region.
Resection 2. The physeal cartilage must be open.
A patients age is an important consideration
En bloc resection of all tumoural tissue is here. In patients who have nearly finished
obligatory in bone sarcomas. Depending on the growing the probability of tumoural cells
size of the lesion, as measured from the having crossed the physis is higher, and it is
previous MR, greater or lesser quantity of bone, more difficult to achieve physeal distraction.
together with adjacent soft tissue, will need to 3. The tumour must not have transgressed the
be removed. physis. Pre-operatively, radiography, arteriog-
In the majority of bone sarcomas, the surgical raphy, CT and particularly MRI can be used to
margin must be wide in order to reduce the risk of demonstrate this.
local recurrence. The surgical treatment recommended for these
Bone resections can be divided into diaphy- tumours depends on the stage of invasion of the
seal resections and articular resections. epiphysis as revealed by MRI (Fig. 4).
Physeal distraction is used in tumours of the
Diaphyseal Resections distal femur, proximal tibia, distal tibia, distal
When the tumour does not affect the articular end fibula, proximal humerus and distal radius. In
of the bone, it is possible to conserve the joint. This locations such as the proximal fibula or proximal
has numerous advantages for the patient. In the femur, physeal distraction is not used.
case of pediatric patients, the advantages are even The presence of a pathological fracture
greater, because the long-term problems of pros- contra-indicates physeal distraction, because the
thetic reconstruction in children can be avoided distraction will occur through the fracture instead
and because conservation of the growth- cartilage of through the growth-plate. In such cases,
makes it possible to avoid many subsequent prob- intra-epiphyseal osteotomy could be used to
lems. Loss of the joint (as occurs, by definition, conserve the epiphysis. However, if the fracture
under any articular resection) can later lead to heals during neo-adjuvant chemotherapy
a significant degree of functional deficit. period, it is still possible to perform physeal
It was for this reason, to avoid loss of the joint, distraction.
that Professor Canadell developed, in 1984, The surgical technique usually consists of two
a technique to allow conservation of the articula- steps [15].
tion and with it conservation of a great part of
growth potential. First Step Two pins are inserted into the epiph-
ysis and another two into the diaphysis, 810 cm
Canadells Technique away from the tumour (Fig. 5). An external
Histological Fundamentals, Image Methods monolateral fixator with a T-shaped piece for
and Indications the epiphyseal pins is attached.
Traditionally, the physis has been regarded as Distraction is begun in the operating room
a barrier capable of preventing tumour extension and continues at the rate of 11.5 mm/day until
in some cases. Such extension relates to both 1 or 2 cm of distraction is achieved. During the
delay in diagnosis and tumour aggresion [15]. first few days nothing happens, but usually
Limb Salvage in Children 4261
Tumor Graft
If this happens, it is still possible to conserve the reconstruction of both the bone and the joint more
epiphysis without increasing the likelihood of difficult.
local recurrence by carrying out an intra- An articular resection implies loss of
epiphyseal osteotomy. the growth-plate adjacent to the joint. Articular
cartilage is usually a barrier to tumour spread, and
Other Techniques for Preserving Joints so resection of the other bone of the joint is not
In selected cases, the epiphysis can be preserved necessary. If the tumour has spread through the
by intra-epiphyseal osteotomy [31] (Fig. 8). We ligaments or capsule, then an extra-articular
believe that intra-epiphyseal osteotomy is indi- resection should be performed.
cated in the following particular situations:
Metaphyseal tumours with no involvement of
the physis in which a pathological fracture has Reconstruction
occurred.
Metaphyseal tumours in contact with part of Reconstruction is not needed after resection
the growth-plate. of bone sarcomas at some locations; tumours of
Metaphyseal tumours without involvement of the patella, proximal and middle fibula, rib,
the physis, but in patients who are close to the clavicle, scapula, zone III of pelvic bone, etc. can
end of growth. be extirpated without provoking large functional
deficits (Fig. 9). In some cases reconstruction can
Articular Resections be useful for aesthetic purposes.
When a tumour involves the epiphysis,
usually the articular edge of the bone must to be Biological Reconstruction
resected. In such cases, the capsule, the extra- and For bone reconstruction we can use auto- or allo-
intra-articular ligaments, the tendinous attach- grafts. Bone grafts have the function of pro-
ments, etc. should be removed. This makes resec- moting osteogenesis and providing mechanical
tion surgery more complicated, and it also makes support.
Limb Salvage in Children 4263
Fig. 7 (continued)
4264 M. San-Julian et al.
Fig. 7 (a) Clinical aspect, X-ray and MRI of an osteo- of the extension of the tumour. (c) Intra-operative image
sarcoma involving almost the whole femur in a 12 year- of the tumour. See the accurate dissection of the vascular
old boy. (b) Epiphysiolysis was performed. In this case, bundle. (d) Resection and reconstruction with an interca-
the proximal pins were placed at the femoral neck because lary allograft (marked with arrows)
Limb Salvage in Children 4265
b c
Fig. 8 (a) Osteosarcoma of the distal femur in contact Reconstruction was carried out with an intercalary allo-
with the full physis. (b) Intra-epiphyseal osteotomy for graft. Epiphysis is free of tumour. Lines shows the
joint preservation in a case of osteosarcoma of the femur. planification of the osteotomy. (c) Functional results
4266 M. San-Julian et al.
Fig. 9 Two cases involving locations that required no reconstruction. (a) Clavicle. (b) Fibula
Limb Salvage in Children 4267
Fig. 10 This patient with a Ewings sarcoma suffered ligaments and the patients potential for growth. The
a pathological fracture. Neo-adjuvant chemotherapy was diaphyseal osteotomy required strong osteosynthesis
very effective and the fracture healed. Epiphysiolysis was whilst the metaphyseal end healed with just two Kirschner
performed in order to preserve the joint, capsule, wires
of the ankle can occur. If a non-vascularized fractures. A small percentage of patients can suf-
graft is employed, a subperiosteal resection fer from these complications (infection or fac-
of the fibula allows for subsequent neoformation. ture) many years after implantation, something
Another possible donor bone is the tibia. that does not occur with autografts.
An anterior-internal cortical window can be
used, and with time the tibia will remodel. Combination of Allografts and Autografts
Finally, the iliac crest is the most common Capanna described a combined reconstruction
donor site. technique in which a vascularized autograft from
After resection of large diaphyseal tumours, it the fibula is embedded within a window in the
may be necessary to resort to allografts. These allograft. In this manner, the best is made of
have excellent or good functionality in 80 % of both the mechanical advantages of the allograft
cases [33] although they are associated with and the biological advantages of vascularized
higher rates of infection, pseudoarthrosis and fibula [34].
Limb Salvage in Children 4269
Fig. 11 A vascularized
fibula including the
proximal edge and its
growth- plate can be used
for reconstruction of the hip
or proximal humerus in
growing children
This increases the risk of infection, because of patients clearly prefer such an approach rather
the increase in the number of hours under than amputation (Fig. 13).
surgery. Limb salvage in foot locations can be done in
Tendon suture. It is important to return stabil- almost all patients, as it does not diminish sur-
ity to musculature as physiologically as possi- vival rates and functional outcomes are in most
ble. Reconstruction of the extensor apparatus cases excellent.
of the knee is fundamental to the provision of a
useful, functional limb and to prevent early
failure of the prosthesis. In these cases, the Post-Operative Care and
patellar tendon can be reinserted into the inter- Rehabilitation
nal calf muscle flap. If graft-bank allografts are
being used, these grafts are usually extracted The scar requires special attention. A surgical
such that a part of each tendon is left, for wound in good condition allows an early start to
example, the insertion of the gluteal tendons, chemotherapy treatment. Usually, early removal
rotator cuffs or patellar tendons. In the case of of staples can result in dehiscence, because
arm reconstruction, a tumour sometimes chemotherapy also delays scar formation. If the
involves the rotator cuff, and reconstruction wound shows any necrotic or dehiscent zones, it
requires part of the deltoid muscle. will need to be followed up more carefully, with
Capsule reconstruction. After tumour-resec- consideration of revision.
tion, osteo-articular reconstructions with pros- If the resection was very extensive, oedema of
theses present a greater risk of subluxation the limb is to be expected, and we must start to
since, the articular capsule and surrounding take anti-oedema measures because oedema
muscle are usually affected. Therefore, the delays scarring.
joint is stabilized with a Trevira tube. Drainage tubes are usually withdrawn within
4872 h.
Tumour Treatment in Special Locations Antibiotic prophylaxis is with third-
Foot generation cephalosporin. There is no universal
Malignant bone tumours located in the foot and protocol; there are guidelines advocating 3 days,
ankle are extremely rare [40]. Ewings sarcoma 3 weeks and even 3 months.
can be located in almost any bone, only 5 % of Rehabilitation should be begun in the imme-
the cases occur in the foot and ankle. Dahlin and diate post-operative period. Irrespective of the
Unni published their experience in 8,542 bone surgery, techniques should be used on the
tumours, only 105 (1.2 %) were located at the distal limbs not compromised by surgery. These tech-
lower extremity. 54 were malignant and almost niques involve active mobilization as a means to
half of them were Ewings Sarcoma [41]. ES of preserve mobility of body segments, maintain
the distal lower extremity has better prognosis good blood circulation and make provision for
than those located in other parts of the body. recovery of motor functions.
Usually, radiotherapy, surgery or both are used With regard to the affected limb, in the
for local control of the disease. Amputation has initial phases, physical measures are used to
traditionally been the most common surgical tech- maintain, as far as possible, optimum levels of
nique. It provides excellent local control, but there musculocutaneous tone and vasomotor function.
is an inevitable loss of function. Less aggressive, In conjunction with the measures described
conservative, surgery can achieve the same above, re-absorption of oedema fluid is
excellent results in terms of survival and local potentiated by gentle manual drainage manoeu-
disease control, whilst maintaining better function vres and stimulation of venous-lymphatic
of the extremity. For reconstruction, allografts or return. At the same time, these procedures
plastic devices can be used. These provide excel- improve the local blood flow so necessary to the
lent functional results in most cases, and almost all processes of scarring and the maintenance of the
4272 M. San-Julian et al.
a b
Fig. 13 (a) Ewings sarcoma in the os calcaneum. Most reconstruction. The Achilles tendon was re-attached to the
surgeons would advise amputation. (b, c) After removing gluteal tendons of the allograft. * shows the greater
the femoral head, a proximal femur allograft was used for trochanter
tone of permanent tendomyoligamentous bone appropriate tissue regeneration times for tissue dis-
structures. placements and the characteristics of the sutures
Together with drainage manoeuvres, move- used. With unstable joints, muscle groups are
ment of the joints above and below the surgically potentiated by means of techniques of directed
affected segment are to be encouraged. Such isometric contraction and electrostimulation in
movements, however, must take into account the order to maintain muscular tone.
stability of the joint that has been operated on and Other techniques, such as hydrotherapy, elec-
the osteosynthesis methodology used. trotherapy, spectrum therapy and masso-
With stable joints, treatment with gentle passive therapy serve to help recovery of both joints
unforced physiotherapy can proceed on the first and muscles in the later stages of recovery, when
day after surgery. Such exercises must respect the the surgical wound displays favourable scarring.
Limb Salvage in Children 4273
Fig. 14 Limb length discrepancy and infection in the allograft prosthesis was implanted. Both complications,
same patient. After removing the implant, a lengthening length discrepancy and infection were solved in a single
of soft tissues was performed and a new longer composite course of action
4274 M. San-Julian et al.
Fig. 15 (continued)
Limb Salvage in Children 4275
Fig. 15 (continued)
How to avoid infection: aseptic and anti- is sufficient. If the subluxation is recurrent,
septic measures must be painstaking (the risk it may be necessary to change the
of infection is up to 130 greater than that with prosthesis. Constrained prostheses should be
arthroscopy). All foreign material used for considered.
reconstruction (whether metallic or biologi- In the hip, large diameter heads can be
cal) must be fully covered by muscle. When used. When such measures are not sufficient,
necessary, use muscle flaps (for example, there are synthetic materials (such as
from the medial gastrocnemius muscle in a Trevira tube) that can be used to surgically
cases of tumours in the proximal tibia). create a neocapsule for the joint.
Antibiotic prophylaxis must be maintained
for sufficient time.
(d) Subluxation/Dislocation. Resection of the Post-Operative Complications
capsule increases the risk of subluxation
despite the fact that tumoural joint prostheses Limb Length Discrepancies
are usually hinged or constrained. Resection As mentioned in previous sections, bone sarco-
of bone together with soft tissues provokes mas in childhood are usually located close to the
greater instability than that encountered with growth cartilage and their resection can result, in
a standard prosthesis placed for a fracture or many cases, in alteration in growth [46]. Limb
for arthritis. length discrepancies in the upper body are less
Treatment. In some cases, reduction limiting in ordinary life and often only result in
together with subsequent immobilization an aesthetic deficiency. In the legs, however,
4276 M. San-Julian et al.
Fig. 15 (a) Limb-length discrepancy after reconstruction (b) Bone lengthening was performed. (c) At the end of
of the distal femur with non-vascularized autograft from growth, this patient still had his knee and was free of
the fibula. Notice the equinus of the left foot the discrepancy
osteosynthesis device did not allow subsequent growth.
a discrepancy greater than 34 cm may impose elongation, for example, when a graft or insert
a real physical limitation. has to be removed due to infection or fracture.
Treatment. There are many options to resolve Elongation can be through the patients healthy
this problem. The simplest treatment is to perform bone, or the reconstruction material can be
a contralateral, percutaneous epiphysiodesis. substituted for a larger version and elongation
But, with this method it is only possible to correct applied through the soft parts. Expandable pros-
a small discrepancy, depending on the age of the theses are designed to be elongated inside the
patient. Another option is to shorten the healthy patient. However, if the length discrepancy is
limb; this, too, is only for small (45 cm) very big, it can be necessary to exchange
discrepancies. the prosthesis.
If the above procedures are not sufficient, How to avoid length discrepancies: when pos-
elongation through external fixation can sible, preservation of the growth cartilage will
be undertaken (Fig. 15). Sometimes, a complica- avert the problem of later discrepancy. For
tion can be made into an opportunity for tumours located at a distance from the physis, if
Limb Salvage in Children 4277
a b c
Fig. 16 (a) Fibrosarcoma of the femur in a 16-year-old (c) A new osteosynthesis device and autologous bone
girl. (b) An intercalary allograft was used for reconstruc- graft supplementation were used to resolve this
tion, but the proximal osteotomy failed to unite. complication
4278 M. San-Julian et al.
12. Wozniak W, Rychlowska M, Kuczabski M, et al. Eval- the extremities or trunk: an analysis of 1,702
uation of indications and the perspectives of limb sal- patients treated on neoadjuvant cooperative osteosar-
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