Abstract
Background: The treatment of congenital pseudarthrosis of the tibia
remains difficult and controversial. The purpose of this study was to evaluate
the long-term results of a technique consisting of excision of the
pseudarthrosis, autologous bone-grafting, and insertion of a Williams
intramedullary rod into the tibia.
Methods: Twenty-one consecutive patients with congenital
pseudarthrosis of the tibia were managed with this technique between 1978 and
1999, and the results were retrospectively reviewed. The mean age of the
patients at the time of the latest follow-up was 17.2 years (range, seven to
twenty-five years), and the mean duration of postoperative follow-up was 14.2
years (range, three to twenty years).
Results: Initial consolidation occurred in eighteen of the
twenty-one patients. Refracture occurred in twelve patients; five fractures
healed with closed treatment, five healed after an additional surgical
procedure, and two ultimately required amputation. Ten patients had an ankle
valgus deformity after tibial union. Eleven patients had a residual
limb-length discrepancy of >2 cm; six required a contralateral distal
femoral and/or proximal tibial epiphyseodesis, two had a tibial lengthening,
and one used a shoe-lift. Five patients had an amputation: two, because of a
recalcitrant fracture; two, because of a limb-length discrepancy (6 and 9 cm);
and one, because of a chronic lower-extremity deformity.
Conclusions: This technique produced a satisfactory long-term
functional outcome in sixteen of twenty-one patients and should be considered
for the management of congenital pseudarthrosis of the tibia.
Level of Evidence: Therapeutic study, Level IV (case
series [no, or historical, control group]). See Instructions to Authors for a
complete description of levels of evidence.
Congenital pseudarthrosis of the tibia is one of the most challenging
problems in pediatric orthopaedics. The natural history is persistent
instability and progressive
deformity1-5.
Treatment options have varied greatly and have included both operative and
nonoperative
approaches6-9.
Although no single method has proven ideal, the highest rates of union have
been reported after
surgery10-16.
Currently, three different surgical approaches—vascularized
bone-grafting, intramedullary stabilization, and external fixation—have
been relatively successful in obtaining initial union of the
pseudarthrosis16-24.
However, despite that
success10,16,18-20,25,
the quality and longevity of the consolidation and the future function of the
involved extremity remain
uncertain15,17,26.
This uncertainty stems from the lack of long-term follow-up studies of the
different surgical methods, particularly the lack of studies identifying the
rates of refracture, limb-length discrepancy, and residual angular deformity.
All of those complications can compromise the functional outcome, even though
the pseudarthrosis may have
healed4,27,28.
Since 1978, we have used a solid intramedullary rod combined with excision
of the pseudarthrosis and autogenous iliac crest bone-grafting for the
treatment of congenital pseudarthrosis of the tibia. This is a modification of
the original technique described by
Charnley29, and it
involves use of the two-part Williams intramedullary solid rod to stabilize
the pseudarthrosis site and transfix the ankle
joint10. Our
preliminary results were reported in
199216. The purpose
of the current study was to both evaluate the long-term results of this
technique and provide an update of our surgical and post-consolidation
strategy when utilizing the transarticular Williams-rod stabilization
technique.
We retrospectively reviewed the results in twenty-one consecutive patients
with congenital pseudarthrosis of the tibia who had been managed with this
technique at the St. Louis Shriners Hospital for Children and the St. Louis
Children's Hospital between 1978 and 1999. The mean age of the patients when
the surgery was performed was 5.1 years (range, eleven months to eleven years)
(Table I). Twelve patients were
girls and nine were boys. All patients had an established pseudarthrosis of
the tibia, which had developed after a fracture in twenty patients and after
an osteotomy in one. The initial fracture occurred at a mean age of 2.5 years
(range, one month to six years). Twelve children had neurofibromatosis, one
had Ehlers-Danlos syndrome, and the remaining eight had no associated
diagnoses. The left tibia was involved in eleven patients and the right, in
ten.
Previous surgery had been performed in eleven patients prior to referral to
our institution. A single compression plate and screws and autogenous bone
graft had been used in five patients; bone-grafting without internal fixation
had been attempted in three patients (Cases 4, 19, and 21); a vascularized
fibular graft and an intramedullary rod had been used in one patient (Case 7);
and an intramedullary rod with autogenous bone graft had been used in another
(Case 3). A pseudarthrosis developed after an osteotomy and fixation with
crossed Kirschner wires for treatment of anterolateral tibial bowing in one
child. Eight of the remaining ten patients had been managed nonoperatively
with casts and/or orthoses. Two patients had had no previous treatment.
Electrical stimulation, either external (four patients) or with an implanted
device (two patients), had been used in six patients as adjunctive
treatment.
The pseudarthroses were classified radiographically with use of Boyd's
system, which includes six distinct radiographic types of anterolateral bowing
deformity1. Three
patients were born with anterolateral bowing and a defect in the tibia (Type
I). Nine pseudarthroses developed in an hourglass constriction of the tibia
(Type II) (Figs. 1-A,
1-B, and
1-C). Six pseudarthroses
developed, without narrowing, at a sclerotic segment of bone (Type IV) (Figs.
2-A,
2-B, 2-C, 2-D2-E), and one of these
pseudarthroses developed after an osteotomy. The remaining three patients had
an attenuated, dysplastic fibula at birth, and a tibial pseudarthrosis
developed (Type V). All but three of the pseudarthroses were located in the
distal third of the tibia.
Anteroposterior and lateral radiographs were made every three months
postoperatively until osseous union was achieved and variably thereafter.
Scanograms were used to measure any clinically evident limb-length
discrepancy. All patients were examined by one of the senior authors at the
time of the most recent follow-up. Particular attention was paid to the range
of motion of the ankle, residual limb-length discrepancy, and any residual
angular deformity at the ankle. The number and type of additional surgical
procedures following our initial procedure were recorded. The mean age at the
latest follow-up examination was 17.2 years (range, seven to twenty-five
years). The mean duration of follow-up after insertion of the intramedullary
rod was 14.2 years (range, three to twenty years). Fourteen patients were
skeletally mature at the time of the latest follow-up.
Treatment Protocol
The same operative technique was used for all patients. The patient is
placed in a semilateral position on a radiolucent operating table with a
support beneath the hip, and a sterile tourniquet is applied to the proximal
part of the thigh. The entire lower extremity and ipsilateral iliac crest are
prepared and draped. An iliac crest bone graft, consisting of adequate
corticocancellous strips and cancellous bone, is obtained first, and the wound
is closed. The hip support is removed, and the patient is positioned supine.
An anterior longitudinal incision is made, extending several centimeters both
proximal and distal to the pseudarthrosis site. The pseudarthrosis is
completely excised until there is normal bleeding bone of the medullary canal
of both the proximal and the distal tibial fragment. The resection shortens
the tibia by 1.0 to 3.0 cm. The medullary canal of both tibial fragments is
reamed with a drill and/or a curet.
The tibia is then stabilized with a Williams rod (Figs.
3-A and
3-B). This rod consists of an
indwelling rod and an insertion rod that either are available commercially
(Zimmer, Warsaw, Indiana) or are custom-made for each patient from a hospital
stock of Kirschner, Rush, and Ender rods. Preoperative planning is essential
for selecting a rod of appropriate length and diameter. The length of rod that
is needed is calculated on the basis of the expected length of the tibia after
the pseudarthrosis has been excised and any angular deformity is corrected.
The rod typically transfixes the ankle joint, and the anticipated remaining
growth determines the appropriate location of the distal end of the rod at the
time of surgery. With longitudinal growth, the distal part of the tibia, the
ankle, and the foot migrate distally. Our current strategy is to minimize the
duration of ankle immobilization by the rod. Usually, the distal end of the
rod should extend well into the body of the calcaneus in children four years
of age or less, whereas it should end in the talus in children five to ten
years of age. Following consolidation, at approximately one to two years after
rod placement, we perform a second operation to push the rod across the ankle
joint. The rod is not placed across the ankle joint when the patient has a
relatively proximal pseudarthrosis or is nine years of age or older.
The entire rod assembly is inserted into the medullary canal of the distal
segment and is advanced with a power source antegrade across the distal part
of the tibia, the ankle joint, and the subtalar joint and out through the heel
pad. During the passage of the rod across the ankle joint, it is imperative
that the foot be positioned to minimize the tendency for calcaneal and valgus
angulation of the distal segment and/or hindfoot. The desired ankle position
is neutral dorsiflexion-plantar flexion, verified clinically and with
fluoroscopy. The tibial segments are anatomically aligned at the
pseudarthrosis site, and the rod is then driven retrograde into the proximal
fragment. Again, direct visualization and the use of fluoroscopy are
imperative to confirm that the rod is within the medullary canal and that the
tibia is aligned in the coronal and sagittal planes. Occasionally, because of
extensive deformity, an additional osteotomy of the proximal fragment is
necessary to ensure both intramedullary passage of the rod and a more anatomic
alignment of the tibia. Unscrewing the insertion rod one turn and noting the
rod junction on fluoroscopy confirms the exact location of the distal end of
the indwelling rod. The insertion rod is removed when an appropriate position
has been achieved.
Fixation of the fibula adds stability to the construct if the fibula is
fractured or if fibular shortening was necessary to achieve adequate
apposition of the tibia. Fibular fixation is often less than optimal because
of the small size of the medullary canal. When stabilization is performed, we
prefer intramedullary fixation with an appropriately sized Kirschner wire,
which is placed into the distal fragment through an open incision after
excision of the pseudarthrosis. The wire is directed antegrade, through the
distal tip of the fibula, and then retrograde into the proximal fragment.
The previously harvested cancellous and corticocancellous bone graft is
then placed around the tibial pseudarthrosis site and is secured with
circumferentially placed absorbable heavy suture material. Syngraft (GenSci
Regeneration Sciences, Irvine, California) is added in children who are four
years of age or less. A suction drain is placed over the pseudarthrosis site.
The deep fascia of the leg is not closed. The subcutaneous tissue and skin are
closed in separate layers.
According to the protocol, children six years of age or less are treated
with a one and one-half spica cast to ensure minimal rotatory stress at the
pseudarthrosis site. The spica cast is replaced with an above-the-knee cast at
six to eight weeks postoperatively. Cast immobilization is discontinued at
approximately four to six months postoperatively, once clinical and
radiographic examination indicates that union has occurred. Older children are
treated with an above-the-knee cast with the knee flexed 30° for an
average of four months. Once the cast is removed, the involved extremity is
protected with a custom-fabricated clamshell knee-ankle-foot orthosis with a
locked ankle joint and a free knee joint. Adequate circumferential leg contact
is an essential feature of this orthosis, which is worn twenty-four hours a
day. Walking with progressive weight-bearing is initiated. For children nine
years of age or older, the knee-ankle-foot orthosis can be converted to a
total contact ankle-foot orthosis as consolidation progresses.
With longitudinal growth of the tibia, the rod remains in place and the
distal part of the tibia and the foot migrate away from its distal end until
the ankle joint is free. This took approximately three years to occur in the
patients treated earlier in our study. Currently, to minimize the duration of
ankle joint immobilization and the potential for disruption of the articular
cartilage during the gradual migration of the rod across the ankle joint, we
surgically advance the rod across the ankle joint, with a pusher rod, when its
distal end approaches the articular surface of the talus. The orthosis is
unlocked at the ankle once the rod lies proximal to the ankle joint. The
orthosis is worn until the patient reaches skeletal maturity and then for
sports activities thereafter.
The technique resulted in initial consolidation of the pseudarthrosis in
eighteen of the twenty-one extremities, at a mean of sixteen months (range,
five to forty-three months) (Figs.
2-A2-B, 2-C, 2-D2-E). Union was achieved in the
three remaining tibiae after a second bone-grafting procedure. Two patients
had both a proximal and a distal tibial osteotomy in order to realign the
tibia at the time of the initial insertion of the rod.
Growth of the tibia resulted in the distal end of the rod being located
proximal to the ankle joint in twelve patients, at a mean of thirty-four
months (range, twenty to fifty-eight months) postoperatively. In three
patients, treated early in the series, the rod continued to traverse the ankle
joint and was removed in an attempt to regain ankle motion; all three patients
sustained a refracture, which required repair of the pseudarthrosis and
reinsertion of the rod (Table
I). An additional three patients, in whom the distal part of the
tibia failed to migrate away from the distal end of the nail, had delayed
union of the pseudarthrosis and were treated with repeat bone-grafting and
replacement of the rod. In those patients, the proximal portion of the
replacement rod was surrounded with methylmethacrylate, through a separate
bone window created in the proximal tibial metaphysis, to help anchor it to
the proximal part of the tibia. With subsequent growth, the distal part of the
tibia and the foot migrated away from the rod in all three patients. Eight
patients in whom the rod was no longer traversing the ankle joint regained
functional motion of the ankle, which we defined as a total arc of motion
(maximum dorsiflexion to maximum plantar flexion) of =25°. Four
patients had nonfunctional ankle motion even though the rod was no longer
crossing the ankle joint, and in one patient the rod was still transfixing the
ankle joint at the time of this review. We purposefully did not transfix the
ankle joint with the Williams rod in three patients; all had a functional
ankle range of motion at the time of final follow-up.
Twelve patients had a refracture after the initial consolidation, at a mean
of 9.0 years (range, 2.5 to 11.7 years) following the initial repair of the
pseudarthrosis. Three refractures occurred after removal of the rod in an
attempt to regain ankle motion. The mean age at the time of the initial
diagnosis of the congenital pseudarthrosis of the tibia was 4.8 years (range,
one to nine years) for the patients who had a refracture compared with 5.5
years (range, one to eleven years) for those who did not have a refracture.
All twelve refractures were initially treated nonoperatively. Five healed with
a combination of cast immobilization, bracing, and external electrical
stimulation. Five others were successfully treated surgically after
nonoperative management had failed. Two of those patients underwent open
reduction and internal fixation with a plate and screws and autogenous iliac
crest bone-grafting, and the remaining three patients were treated with
revision intramedullary nailing and autogenous bone-grafting. Two patients
with recurrent fractures did not have healing and underwent amputation.
Ten patients had a fibular pseudarthrosis at the time of the intramedullary
nailing of the tibia. Four of these patients were treated with excision of the
fibular pseudarthrosis and intramedullary fixation of the fibula, two had
excision of the pseudarthrosis alone, and the remaining four patients had no
fibular surgery. Two of the six patients treated with excision of the fibular
pseudarthrosis with or without internal fixation had a refracture of the tibia
compared with two of the four patients who had a fibular pseudarthrosis but no
fibular surgery. There was a trend toward increased ankle valgus deformity
postoperatively in the patients who had had a fibular pseudarthrosis
preoperatively (six of ten patients), even when the fibular lesion had been
excised and had subsequently united. Only four of the eleven patients who had
not had a fibular pseudarthrosis preoperatively had a clinically relevant
ankle valgus deformity postoperatively. Of the eleven patients who had an
intact fibula at the time of surgery, eight had a fibular osteotomy to aid in
the alignment of the tibial fragments; of these eight patients, six had a
refracture of the tibia, and three of the six patients eventually had an
amputation. Of the three patients in whom an intact fibula was not treated
with an osteotomy, two had a refracture of the tibia but no amputations were
required.
Eleven patients had a clinically relevant limb-length discrepancy,
averaging 5 cm (range, 2 to 9 cm). Six of these patients were treated
definitively with a contralateral distal femoral and/or proximal tibial
epiphyseodesis, with a mean predicted limb-length discrepancy of 4 cm
(Table II). Two patients were
treated with tibial lengthening with the Ilizarov method, with the corticotomy
for the lengthening performed in the proximal part of the tibia. One patient
with a 3-cm discrepancy was treated with a shoe-lift. Two other patients, with
6 and 9-cm limb-length discrepancies and associated ankle valgus deformity,
underwent amputation. Of the remaining ten patients, who had a limb-length
inequality of <2 cm, three were still skeletally immature at the time of
writing.
Anteroposterior weight-bearing radiographs of the ankle revealed an ankle
valgus deformity, after consolidation of the pseudarthrosis and growth of the
distal part of the tibia to free the ankle joint, in ten patients. In nine
patients, the valgus deformity was successfully treated with medial distal
and/or proximal physeal stapling of the tibia at a mean age of eleven years
(range, eight to fourteen years). After the valgus deformity had been
corrected, the staples were removed and a syndesmosis screw was inserted in
the six patients in whom an ununited distal fibular fragment remained. This
allowed symmetric growth of the distal part of the tibia without recurrence of
the valgus deformity. Two syndesmosis screws migrated proximally and three
broke (Fig. 1-C), but only one
has been surgically removed to date. The remaining patient with a valgus
deformity was treated with a proximal tibial osteotomy at the age of thirteen
years.
Five patients required amputation because of chronic lower-extremity
dysfunction: two, because of recurrent fracture nonunion; two, because of
limb-length discrepancy; and one, because of residual angular deformity. Three
of the five patients had a below-the-knee amputation, and the other two
initially had a Syme amputation. However, the two Syme amputations were
revised to below-the-knee amputations because of instability of the stump and
difficulty with prosthetic fitting. All five patients who had an amputation
were fully weight-bearing with the use of a standard below-the-knee prosthesis
for walking at the time of follow-up. There were no deep infections,
neurovascular complications, or broken rods.
Currently, three surgical techniques (vascularized fibular grafting,
intramedullary stabilization, and external fixation) are being used with
relative success for the treatment of congenital pseudarthrosis of the
tibia16-23.
However, there is a lack of long-term studies of these methods. As Boyd
suggested years ago, the true success of treatment of congenital
pseudarthrosis of the tibia in a growing child can be known only by following
the patient until
maturity1.
The current technique of free vascularized fibular grafting has been well
described24, and
several authors have reported success in obtaining initial tibial
consolidation15,17,24,30-32.
However, there are many potential pitfalls. A secondary bone-grafting
procedure is frequently necessary to obtain union at the pseudarthrosis
site24. In
addition, refracture and recurrent nonunion at one end of the graft
site17,24,
or in the graft itself
31, are not
uncommon following consolidation. Another problem is malalignment—either
anterior bowing or valgus deformity—of the affected
tibia24. These
angular deformities do not remodel and often are
progressive15,24.
In addition, progressive valgus ankle deformity on the donor side with
proximal migration of the distal part of the fibula is a problem following
harvest of a vascularized fibular graft in
children31,33.
To prevent this complication, tibiofibular metaphyseal synostosis (the
Langenskiöld procedure) has been
recommended24,33.
However, in a recent review, this procedure was found to only delay, not
prevent, the development of ankle valgus in children after harvesting of a
vascularized fibular
graft33.
Numerous authors have reported on the use of a circular external fixator in
the treatment of congenital pseudarthrosis of the
tibia20,26,34-36.
Fifteen patients with a total of sixteen congenital pseudarthroses of the
tibia treated with the Ilizarov apparatus were reported on by Paley et
al.20. Initial
union occurred in fifteen of the sixteen extremities after one treatment and
in one extremity after one additional treatment. The mean age at the time of
treatment was eight years compared with a mean age of five years at the time
of the initial rod placement in the current series. Five refractures occurred
in the series by Paley et al., and all were successfully treated with a
variety of additional procedures. In a more recent study, Boero et
al.26 reported
failure of the Ilizarov technique in eight of twenty-one patients with
congenital pseudarthrosis of the tibia. Consolidation was obtained in twelve
of fourteen patients who were operated on at the age of five years or older
and in only one of seven patients who were treated at the age of four years or
younger.
Umber et al.10
popularized the use of the two-part Williams intramedullary solid rod in North
America. We reported our initial results with the use of this technique in
199216. Although
union was obtained in all ten patients at a mean of six years postoperatively,
we observed problems in many patients, including failure of distal tibial
migration, stiffness of the ankle, limb-length discrepancy, and residual
angular deformities at the ankle. In the interim, we have continued to follow
these patients, added others to our series, and modified the technique to
improve our results.
Despite reports of ankle stiffness following long-term transfixation of the
tibiotalar and subtalar
joints1,37,
only a few patients in our study did not regain functional ankle motion once
the rod no longer transfixed the ankle joint. In order to minimize loss of
ankle motion, we now recommend surgically advancing the rod out of the
hindfoot and ankle joint soon after the pseudarthrosis has healed (usually
less than two years after rod insertion). The initial choice of rod length is
crucial to ensure that, at the time of rod advancement, it is not pushed
across the proximal tibial physis and/or into the knee joint. In general, if
the ankle joint is going to be transfixed for less than two years, the rod
should be initially placed no less than 2 cm from the proximal tibial physis.
In cases in which the pseudarthrosis is more proximal and/or the patient is
nine years of age or older, intramedullary fixation should be obtained without
transfixing the ankle joint.
In an attempt to decrease the prevalence of ankle stiffness, custom
interlocking intramedullary nails that do not transfix the ankle joint were
developed and are now available; they may be appropriate for patients in whom
the segment distal to the pseudarthrosis is large enough to accommodate
adequate
fixation38.
Unrestricted ankle motion, however, may allow the distal tibial segment to
move sufficiently to delay or even prevent union of the pseudarthrosis.
Another disadvantage of locked nails is that even custom nails are too large
for use in many younger children. The solid two-part Williams intramedullary
rod can be used in children of all ages and has distinct advantages in terms
of strength and the ability to provide rigid immobilization and protect the
consolidating pseudarthrosis. In the current series, temporary transfixation
of the tibiotalar and subtalar joints did not negatively affect the long-term
functional result.
Failure of the tibia to migrate distally from the rod was another problem
in our patients. Early in the series, this was treated by simply removing the
rod, but refracture soon occurred in all three patients. Currently, failure of
the tibia to migrate distally is treated with reinsertion of a rod at the
initial pseudarthrosis site. The replacement rod is secured to the proximal
part of the tibia with methylmethacrylate applied to its proximal 2 cm through
a cortical window. Fixing the rod proximally facilitates migration of the rod
through the hindfoot with growth of the distal part of the tibia.
Refracture is common in patients with congenital pseudarthrosis of the
tibia, despite apparently solid clinical and radiographic
union16,39-41.
This complication occurred in twelve of our patients. Three of the refractures
occurred early in the series, after removal of the rod in an attempt to regain
ankle motion. We agree with others who have concluded that it is inadvisable
to remove the rod after
union10,16,29,42.
An attempt was made to identify risk factors for refracture in this patient
population. The twelve patients who had a refracture were younger at the time
of the initial fracture than those who did not have a refracture. This finding
is consistent with the findings of Roach et
al.43 as well as
those of Kim and
Weinstein44, who
stated that late-onset fracture and pseudarthrosis is a more benign form of
the lesion. However, with the small numbers of patients in our series, neither
the presence of associated disorders (neurofibromatosis and Ehlers-Danlos
syndrome) nor the Boyd type of the pseudarthrosis had significance in terms of
predicting the prevalence of refracture.
The need for fibular surgery remains controversial.
Johnston38
concluded that it is crucial to resect a fibular pseudarthrosis or, if the
fibula is intact, to perform a fibular osteotomy in order to achieve optimal
limb alignment and union. Similarly, in our series, the prevalence of tibial
refracture was higher in the patients in whom the fibular pseudarthrosis was
not resected (two of four) than in those who had a resection with or without
internal fixation (two of six). However, unlike Johnston, we found no
difference in outcome between the patients in whom an intact fibula had been
osteotomized and those in whom an intact fibula had been left alone. The
numbers of patients were too small for us to be able to draw conclusions on
the efficacy of performing an osteotomy of an intact fibula.
Valgus deformity of the ankle can compromise the functional result in
patients treated successfully with an intramedullary rod. We have speculated
that the valgus deformity is more a natural outcome of the initial deformity
(deficient fibula and lateral support) and less an iatrogenic complication of
traversing the tibial physis with the rod since the rod is positioned in the
central portion of the physis. In our study, the majority of the ankle valgus
deformities occurred in patients with a concomitant fibular pseudarthrosis,
even when the fibular lesion had been treated and had subsequently united.
Early in the series, we noted a tendency toward recurrent ankle valgus
deformity in patients with a united fibular fragment and deficient lateral
support. To prevent this complication, we now recommend placement of a
syndesmosis screw once valgus correction has been achieved in patients with a
deficient fibula. This technique has maintained correction in all six of our
patients so treated.
Limb-length discrepancy is common after treatment of congenital
pseudarthrosis of the tibia and was clinically relevant in half of our
patients. It is primarily the result of chronic resorption of bone at the
pseudarthrosis site prior to repair and secondarily as a result of the
operative resection of the pseudarthrosis. We found that most cases can be
treated successfully with an appropriately timed contralateral distal femoral
and/or proximal tibial epiphyseodesis, a tibial lengthening procedure, or a
shoe-lift.
Most cases of congenital pseudarthrosis of the tibia can be successfully
treated with the modified Williams technique, but attention to detail is
critical. On the basis of our twenty-four years of experience with this
technique, we can make several recommendations. The essential surgical steps
of the technique include complete resection of the tibial pseudarthrosis,
intramedullary stabilization of the tibial and if possible the fibular
pseudarthrosis, and iliac crest bone-grafting. In a young child with a typical
distal tibial deformity, the intramedullary rod should extend into the
hindfoot to temporarily immobilize the subtalar and ankle joints; the desired
ankle position is neutral dorsiflexion-plantar flexion. For less common, more
proximal lesions and/or in older children, the intramedullary rod should
extend only to the distal tibial physis. A fibular pseudarthrosis, if present,
should be resected and intramedullary fixation should be attempted with an
appropriate-sized Kirschner wire; an intact fibula should be osteotomized if
shortening appears to be necessary to achieve adequate apposition of the
tibial fragments. Although consolidation typically occurs following this
treatment, refracture, failure of the rod to migrate, shortening, and ankle
valgus deformity are typical problems. In many cases, these problems can be
treated successfully with additional surgical procedures that spare the limb.
Occasionally, despite aggressive surgical treatment, amputation is required
because of chronic lower-extremity dysfunction, as occurred in five patients
in our series. However, the Williams technique produced a satisfactory
long-term outcome in sixteen of our twenty-one patients and should be
considered as the initial operative procedure for congenital pseudarthrosis of
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