Limb Reconstruction Surgery for Fibular Hemimelia By: Dror Paley, MD [email protected]
Fibular Hemimelia is the most common lower extremity congenital
longitudinal deficiency (frequency 1/40,000 live births). . It is associated
with a constellation of deformities including foot ray deficiencies, subtalar
coalition, ball and socket ankle joint, ankle joint malorientation, diaphyseal
angular deformity apex anteromedial, fibular deficiency. .
Congenital shortening of the tibia associated with congenital fibular
deficiency is referred to as fibular hemimelia.
The best known classifications of fibular hemimelia focus on the degree of
fibular deficiency (1,2,4,10) . Most of these classifications were designed at
the time where amputation and prosthetic limb equalization were the only
reliable treatment (15). They are descriptive of the fibular pathologic
abnormality, which does not require reconstruction, and they lack
description of the tibial pathologic abnormality, which is the focus of
reconstruction. Furthermore, they do not help in planning reconstruction of
the foot. Severe rigid and intractable equino-valgus deformity of the foot in
combination with a limb length discrepancy has been the limiting factor for
a successful outcome and the primary indication for ablative surgery. There
have been concerns about inhibition of the tibia growth after lengthening (ref
Paley classification of fibular hemimelia
Type 4: Fixed equino-varus ankle (clubfoot type)
The Type 3-fixed equino-valgus ankles are subdivided into 4 types
according to ankle-subtalar pathoanatomy.
Type 3a-ankle type: the ankle joint is maloriented into procurvatum and
Type 3b-subtalar type: the subtalar joint is has a coalition which is malunited
in equino-valgus with lateral translation
Type 3 c-combined ankle and subtalar: combination of the ankle and
Type 3d-talar type: malorientation of the subtalar joint.
Surgical technique for type 3 ankles: THE SUPERANKLE PROCEDURE (developed by Dr. Paley in 1996)
Under tourniquet control, a longitudinal lateral incision is made parallel to
the posterior cortex of the tibia and is extended distally to the calcaneus. At
the distal end care should be taken not to cut the sural nerve. The distal
sensory branch of the superficial peroneal nerve is identified and dissected
free. Proximal dissection should not be done before finding this nerve. It
runs obliquely across the operative field as it exits the deep fascia. The
peroneal tendon(s) are identified, and if both are present, they are sutured
together proximally and distally and and the longus cut proximally and the
brevis distally. If only one peroneal tendon is present a z-lengthening of this
tendon is performed. The fibrous peroneal tendon sheath is excised. The
distal fibular cartilaginous anlage, if present, is dissected free from all except
a distal flap of soft tissue. It is then reflected distally on this calcaneo-fibular
ligamentous pedicle to expose the ankle joint capsule. The fibrous fibular
anlage is in continuity with the interosseous membrane. Both are dissected
to the level of the apex of the diaphyseal procurvatum. .The interosseous
membrane is resected up to this level but the fibrous fibular anlage should be
removed along its entire length. The proximal part of the fibrous anlage is
exposed using a second incision at the proximal tibia. The second incision is
made parallel to the oblique course of the peroneal nerve. The peroneal
nerve is identified, decompressed and dissected free of the anlage. The
fibular anlage is then dissected from proximal to distal to communicate with
the distal dissection. The anlage is dissected free from the overlying
muscles and the interosseous membrane by tunneling between the two
incisions. It is then detached proximally and removed as a single unit
distally. . The anlage is preserved in saline solution for later use. The
interosseous membrane distally is also resected The Achilles tendon is then
exposed. Before lengthening this tendon the posterior tibial nerve should be
identified. It is located immediately anteromedial to the Achilles. The
posterior tibial neurovascular structures should be dissected free from the
Achilles and then a Z-lengthening of the Achilles tendon is performed,
leaving the distal attachment medial if possible. If the tendon inserts laterally
and the muscle medially, Z lengthen the tendon and leave it attached
laterally distally. Lengthen the Achilles tendon approximately 4 cm in an
infant. Identify and resect all fascial bands inserting onto the dorsum of the
body of the calcaneus. The fascia surrounding the neurovascular bundle
should be freed from its posterior attachment to the calcaneus. This
The ankle level is identified but the ankle capsule does not need to
intentionally be opened. A small opening in the capsule may occur when the
cartilaginous anlage is reflected. An ankle arthrogram can be performed to
visualize the orientation of the distal tibial articular surface. If the ankle
joint surface is laterally and posteriorly oriented. the equino-valgus should
be corrected by supramalleolar osteotomy (Type 3a). To prevent recurrent
deformity it is important to pin the ankle and subtalar joints in the position
of equino-valgus. Two 1.5mmK-wires are drilled into the calcaneus and
across the talus and ankle joint stopping at the distal tibial growth plate.
Using an osteotome or a saw a supramalleolar osteotomy is performed from
posterolateral to anteromedial. It should converge on the anteromedial distal
tibial physis. An acute opening wedge is performed in this oblique plane
hinging on the anteromedial physis. A wedge shaped bone graft
(autogenous iliac crest or allograft; our preference is adult fibular allograft)
is inserted into the opening wedge defect. The K-wires are advanced across
the osteotomy and graft into the diaphysis of the tibia. The foot should only
be corrected to make the plantar aspect perpendicular to the distal tibial
If the ankle joint orientation is near normal but a subtalar coalition with
lateral translation and valgus is present (type 3b), the osteotomy is made
through the coalition of the posterior facet of the calcaneus. This osteotomy
is made obliquely from superolateral to inferomedial. To displace this
osteotomy the calcaneus should be levered distally to correct the equinus and
foot height. Medial translation usually occurs automatically. . Although it is
preferable to make this cut through bone, it may be necessary to do this
through cartilage if there is no ossification of the calcaneus adjacent to the
coalition. . The subtalar coalition osteotomy should not be into the neck of
the talus. It ends in the sinus tarsi. This osteotomy is fixed with two K-wires
from the calcaneus into the distal tibia. In some cases there is both a mal-
orientation of the ankle joint and a malunion of the subtalar coalition (type
3c). In such case both the supramalleolar and the subtalar osteotomies
In some cases instead of a coalition there is a hypermobile unstable subtalar
joint (type 3d). This is due to increased inclination of the subtalar posterior
facet. An arthrogram of the subtalar joint can be performed to confirm this
deformity To correct this type of valgus deformity, the talus is osteotomized
proximal and parallel to the posterior facet. This osteotomy does not extend
into the neck of the talus. Reorienting the subtalar joint maintains subtalar
joint mobility. The opening wedge is fixed with two K-wires. A bone graft is
After the osteotomy realignment is completed the cartilaginous fibular
anlage if present is sutured with absorbable suture to the distal tibial
epiphysis such that it abuts against the talus. The Achilles tendon and
peroneal tendon(s) are repaired in a Z fashion. In most cases the Achilles
tendon repair is end to end with no overlap due to the amount of equines that
was corrected. The fibrous fibular anlage that was previously excised is
sutured across this repair to help prevent disruption during lengthening. If
the Achilles tendon is only laterally attached to the calcaneus, the anlage can
be extended and sutured to the medial calcaneus. The incisions are then
closed. During the procedure the tourniquet remains inflated only for the
first hour and a half. The rest of the procedure is performed with the
The only deformity remaining to be corrected is the diaphyseal one.
This can be corrected acutely by removal of a wedge or trapezoidal segment,
or gradually with a percutaneous osteotomy and application of an external
The Ilizarov fixator is applied, and the lengthening osteotomy is
performed. If there is a diaphyseal procurvatum deformity, the lengthening
osteotomy should be at its’apex. If there is no diaphyseal angulation, the
lengthening osteotomy should be more proximal. Lengthening begins 4 days
after surgery at a rate of 0.8 mm per day with the pediatric Ilizarov device in
children under age six and 1 mm per day with the standard Ilizarov rings for
older children and adults. If there is a diaphyseal deformity, it is corrected
gradually, simultaneous with the lengthening. This usually requires 0.5 mm
per day distraction at the two hinges and 2 mm per day at the posterior
distraction rod. In toddlers, no more than 5 cm of lengthening is performed
to avoid growth inhibition. If there is an internal rotation deformity, the
frame is modified and the leg gradually rotated externally. In children
younger than 8 years, hinges are applied to varusize the tibia to compensate
for the expected valgus drift. Varusization and complete fibrous fibular
excision are two recent modifications that were added to the treatment
protocol after the results from this study were analyzed. More recently we
prefer to use the Taylor Spatial Frame (Smith and Nephew Orthopedics,
Memphis, Tenn.) because of its ability to perform angulation, length,
rotation and translation without laborious time consuming modifications of
DISCUSSION
Lengthening reconstruction surgery for fibular hemimelia can be
divided into two steps: foot deformity correction and limb length
equalization. In the absence of foot deformity, limb lengthening can be
performed in one or more stages depending on the amount of lengthening
required. Most cases are treatable with one, two, or three lengthenings (7). If
the foot deformity of fibular hemimelia is present without limb length
discrepancy, the treatment consists of soft tissue and bone procedures to
correct the deformity. Because the deformity is in a growing child, some
recurrence rate is expected, as with clubfoot. When foot deformity is present
in association with limb length discrepancy, the techniques used to correct
foot deformity and the methods used for limb length equalization are
Surgical releases for clubfoot correction have evolved from the Turco
posteromedial release to the circumferential Cincinnati approach. The
correction of equinovalgus for fibular hemimelia has also undergone
evolution. Gradual distraction of the foot deformity, in our experience, led to
a high rate of recurrence and degenerative changes. Extra-articular soft
tissue releases (includingAchilles tendon, peroneal tendons, fibrous anlage,
interosseous membrane remnant, and fascial bands) led to some
improvement in foot position but was frequently associated with residual
dynamic valgus deformity. Extra-articular soft tissue release combined with
intra-articular release (posterolateral or circumferential ankle arthrotomy)
provided a better correction but still led to recurrence and/or stiffness.
Recurrence is not surprising, considering that soft tissue release does not
address malorientation of the tibial ankle joint surface. The tibial plafond is
oriented laterally and posteriorly, producing valgus and procurvatum
deformity. This can be seen preoperatively using magnetic resonance
imaging or arthrography. In older children in whom ossification of the distal
tibial epiphysis has already occurred, direct measurement using the
malorientation test for the ankle (measuring the anterior distal tibial angle on
the lateral radiograph [normal range = 80–82] and the lateral distal tibial
angle on the anteroposterior radiograph [normal range = 87–92]) objectively
shows how much of the equinus and valgus is due to ankle joint
malorientation (28). In our study, this was confirmed either by arthrography
or by direct observation at the time of arthrotomy. More recently, we have
begun using magnetic resonance imaging to visualize the cartilage
orientation of the distal tibial articulation and the relationship between the
Realizing that the ankle joint was maloriented led to increasing use of
supramalleolar osteotomy in the latter patients of this study. One
shortcoming of supramalleolar osteotomy is that after the ankle joint is
reoriented, the distal tibial growth plate may be maloriented. Theoretically,
this could lead to gradual recurrence. In practice mild recurrence of the
valgus is occasionally seen and can be addressed at the time of the next
staged lengthening. One way to avoid this theoretical problem is to perform
an acute intraepiphyseal opening wedge osteotomy. Since the realignment is
distal to the growth plate it does not malorient the physis. The limitation of
the latter technique is the size of epiphysis. In most cases, it is too small at
two years of age to perform and intraepiphyseal osteotomy.
The best treatment of foot deformity is a combination of soft tissue
lengthening of the Achilles tendon and peroneal muscles and resection of the
interosseous membrane, aberrant fascial bands, and fibrous fibular anlage
combined with supramalleolar, subtalar coalition, and/or talus osteotomy to
correct valgus and procurvatum. There is no need to resect any cartilaginous
anlage. Instead, it can be moved distally and sutured to the distal tibial
epiphysis to add to ankle stability or else discarded. Equinovalgus foot
correction can be performed separately or in combination with lengthening.
We prefer to combine it with lengthening because the soft tissues are loosest
after release and, therefore, the lengthening is easier and less painful because
muscle tension is reduced. An argument can be made that preservation of
ankle motion might be better if the two procedures are staged. Furthermore
this would permit foot deformity correction at an earlier age, followed by
staged lengthening. Certainly this is the approach we take with the superhip
Biomechanics of Fibular Hemimelia foot Deformity Correction
In the normal ankle joint, the ground reaction force vector passes
antero-medial to the ankle at the time of peak loading in single leg stance.
The lateral path of this force vector is a product of the lateral offset of the
calcaneus to the talus and ankle joint. This produces a lateral moment arm
that is countered by the posterior tibial tendon. It also produces a lateral
displacement force on the talus which is countered by the lateral malleolus.
It is well recognized that even small amounts of shortening or lateral
translation of the lateral malleolus lead to a lateral shift of the talus in the
mortis (Yablon). Valgus tilt of the ankle plafond increases the valgus
moment arm and force of lateral translation. With a combination of valgus
of the plafond, lateral translation of the calcaneus and an absent fibula the
ankle joint is potentially unstable and will eventually sublux or dislocate
laterally especially if the extra-articular muscular forces increase due to
Rationale of Osteotomy Realignment for Fibular Hemimelia
In fibular hemimelia the ankle joint is usually ball and socket. If one
considers the tibial plafond like an acetabulum, the talus is only covered
medially in such ankles. Varus osteotomy covers the talus. More
importantly, supramalleolar varus osteotomy, medially displaces the entire
foot including the calacaneus. The ground reaction vector is moved
medially. The valgus moment arm on the ankle is reduced or eliminated.
Despite the absence of the lateral malleolus there is no tendancy for the talus
to shift laterally. Similarly, when there is a lateral translation malunion of a
subtalar coalition the valgus moment arm is increased. Without a lateral
malleolus the foot wants to rest in valgus and the talus wants to shift
laterally. Medial translation of the calcaneus reduces the moment arm. In
more severe cases a combination of medial calcaneal shift with
supramalleolar osteotomy is required to fully reduce the valgus moment arm.
Weber described a lateral malleolarplasty to treat fibular hemimelia. This
does not reduce the valgus moment on the ankle. As discussed above, the
lateral malleolus is not essential to the function of the ankle if the valgus
moment is reduced. We see no indication for lateral malleolarplasty since it
does not address the rest of the pathoanatomy of the equino-valgus
Finally, genu valgum also contributes to the valgus moment arm on
the ankle. Therefore despite complete correction of the foot deformity, if the
knee valgus is not corrected, the foot deformity may recur. It is therefore
important to determine if the valgus knee is due to deformity of the distal
femur, proximal tibia or both. Correction of these can be carried out using
osteotomy or more simply by means of temporary hemi-epiphysiodesis. The
latter is accomplished either by means of Blount’s staples or Peter Steven’s
Eight Plate (Orthofix, Bussolongo, Italy).
Lengthening Reconstruction Surgery vs Amputation
Most authors agree that lengthening is the preferred treatment for
patients with mild to moderate leg length discrepancy with mild foot
deformities (Paley types 1 and 2). The controversial cases are those that
include more severe foot deformities (Paley type 3, a−c) and greater leg
length discrepancies due to more severe tibial inhibition or combined
femoral and tibial discrepancy. Syme’s or Boyd amputation has been the
conventional recommendation for these more severe cases.
The justification for amputation for the more severe cases has been
the failure of most authors to obtain satisfactory results after limb
lengthening. No one would dispute that amputation with prosthetic fitting
requires fewer surgical interventions, requires fewer days of hospitalization,
and is associated with a lower complication rate. Furthermore, no one would
dispute that with availability of modern prosthetics, limb length equalization
with excellent function can be achieved reliably in patients who have
undergone Syme’s or Boyd amputations (3,13,15). This does not prove,
however, that the best treatment for severe cases of fibular hemimelia is
amputation with prosthetic fitting. Excellent function could also be obtained
if amputation and prosthetic fitting were used as the treatment of clubfoot,
ankle arthritis, or other disabling foot conditions. This is a testimony to the
excellence of modern prosthetics and nothing more.
The challenge, therefore, is not to compare the function achieved in
cases of Syme’s or Boyd amputation with that achieved in cases of
lengthening but rather to improve the results of lengthening. Why are the
results that are reported by many authors so poor? Is it because these cases
are unreconstructable or is it because of fundamental errors in the treatment
strategy? We think the latter is the case. An analysis of the unsatisfactory
results reported in different series in the literature clarified that the
overriding factor associated with poor results is recurrent or residual foot
and tibial deformities. It is not the inability to obtain equalization of limb
length. The few series in which good results were obtained, even in severe
cases of fibular hemimelia, reported that the final result was a stable
plantigrade foot (7,16,22). The total amount of discrepancy can always be
equalized by serial moderate-sized lengthenings rather than by one very
large lengthening. The foot deformity can be treated by various methods,
including soft tissue and bone procedures. If these fail, ankle arthrodesis is a
very successful way of permanently stabilizing the foot (7). It is clear that
ankle arthrodesis should not be the indication for amputation. Therefore,
because the worst-case analysis in stabilizing and correcting the foot
deformity is ankle arthrodesis , there is no reason that the foot cannot be
Johnson and Haideri (20), using gait analysis, showed that patients in
whom lengthening has resulted in plantigrade feet and well-aligned tibiae
have better ankle push-off strength and better knee flexion strength than do
patients who have undergone Syme’s amputation. They noted that the
lengthened limb, even if it was stiff and weak, was less different from its
opposite normal limb than was the prosthetic side in cases of Syme’s
amputation, as compared with its opposite normal limb. They reported that
the lengthened limb with a plantigrade foot was “clearly more functional
Naudie et al. (25) achieved satisfactory results in only four of 10 cases
after lengthening. They compared their group with an amputation group and
concluded that amputation was preferable to lengthening. The reason for the
unsatisfactory outcomes was residual or recurrent foot and tibial deformities.
Cheng et al. (8), in a small prospective group of four lengthenings, had the
same experience with unsatisfactory results secondary to recurrent tibial and
foot deformities. Both groups succeeded in achieving the limb lengthening
amounts desired using the Ilizarov apparatus. These results using the Ilizarov
apparatus are not much different from those reported by Choi et al. (9), who
used the older Wagner method. All of the cases of higher grades of fibular
hemimelia had unsatisfactory results in the study presented by Choi et al.
These unsatisfactory results were attributed to rigid uncorrected
equinovalgus deformity of the foot. They achieved satisfactory results in all
except one of the patients with mild fibular hemimelia, a patient who had a
rigid equinovalgus foot. They, too, concluded that the more severe grades of
fibular hemimelia are not candidates for lengthening surgery and would be
best served with amputation and prosthetic fitting.
After reviewing these reported results, we think that although the limb
length can be successfully corrected in most patients, if the foot deformity is
left uncorrected initially or after recurrence, the final functional outcome
will be unsatisfactory (6,14). This conclusion is also valid for the treatment
of clubfoot and vertical talus deformities.
If one examines the few series in the literature that report good
functional results after limb lengthening, the predominant difference is that
in the final result, not only was the leg length discrepancy addressed
successfully but the foot deformities were also addressed successfully.
Miller and Bell (22) reported the outcomes of 12 lengthenings in cases of
fibular hemimelia. At the time of final follow-up, all limbs had regained full
knee motion and all feet were plantigrade. All except three limbs had
regained their preoperative range of ankle motion. None of the ankles had
residual instability. Despite these excellent final results, 25 complications
occurred in 12 lengthenings. These patients required eight secondary
procedures to treat and correct complications.
Gibbons and Bradish (16) lengthened 10 tibiae in cases of fibular
hemimelia. In all cases, the desired lengthening was achieved and all
patients were able to wear normal shoes without orthoses. A plantigrade
position was achieved in all feet without persistent ankle instability.
Complications occurred in nine of the 10 cases. These were all resolved
either surgically or nonsurgically. Several required foot deformity correction
Perhaps the largest series in the recent literature with the longest
follow-up duration is that presented by Catagni and Guerreschi (7). Using
the modified Dal Monte classification (12), they reported 32 grade 1, 37
grade 2, and 20 grade 3 cases of fibular hemimelia that were treated with
lengthenings, all of which led to completed reconstruction. Of the 32 grade 1
cases, 31 required only one lengthening each and one required a second
lengthening. Equal leg lengths with a plantigrade foot were achieved in each
of these patients. In the grade 2 group, five patients required three
lengthenings each, nine required two lengthenings each, and 23 required one
lengthening each. Thirty-five of the patients each ended the reconstruction
with a plantigrade, functional foot. Two have residual valgus deformity and
require shoes with orthoses. None have undergone ankle arthrodesis. Thirty-
two of the 37 participate in recreational sports, and five limit their activities
as a result of knee stiffness or instability. In the grade 3 group, two required
six stages of reconstruction (a stage referred to as a lengthening or a
deformity correction), four required five stages, six required four stages,
three required three stages, four required two stages, and one required one
stage. Eight of these 20 underwent foot deformity correction as a separate
procedure before the age of 3 years. Sixteen of the feet are plantigrade,
stable, and asymptomatic. Five have residual valgus with stiffness and
require an orthosis to alleviate the symptoms. Although most of the patients
could bike or swim, athletic pursuits were more limited than in the grade 1
and 2 patients. There were no permanent sequelae of knee subluxation, hip
subluxation, nerve injury, nonunion, or osteomyelitis in any patient. All of
the patients were satisfied with the functional results of their reconstruction.
Our results are similar to those of these last three studies. We were
able to achieve good or excellent functional results, including the desired
goal of lengthening, in 36 of the 38 legs lengthened. The one patient who
was rated as having achieved only a fair results was lost to followup and had
a residual equinus deformity with a painful arthritic ankle. This patient had
an ankle fusion at another center but we were not able to evaluate the final
result. Many of our patients are involved in recreational and/or competitive
athletics. All of the adults in our series are gainfully employed, including the
one with a fair result due to ankle arthritis. We, too, experienced a high
complication rate, and our study (as did the last three studies mentioned
above) showed that the final result is not related to the complication rate.
Few of the complications lead to major sequelae; those that do can usually
One of the other criticisms of lengthening is the psychologic impact
on the child. Although lengthening is undisputedly stressful for the child and
the family, two recent studies have shown that the majority of the problems
were transitory and remitted with appropriate treatment (17,23). The
lengthening treatment did not cause long-term psychologic maladjustment
(17). Although most patients in our experience tolerated the lengthening
process well, some patients did develop loss of appetite, weight loss, and
difficulty sleeping. We have found that a single small dose of amitriptyline
before bed time is useful in helping these patients. Contrary to popular
belief, lengthening should not be an excruciatingly painful experience. If a
patient is complaining of a lot of pain, especially during the day while at
rest, the cause of the pain should be sought. Pain may be related to pin
infection, pin loosening or cutting out, frame instability, nerve stretch, reflex
sympathetic dystrophy, rupture of the regenerating bone after premature
consolidation, etc. Appropriate treatment, such as antibiotics, pin removal,
wire retensioning, slowing distraction, replacing pins, and backing up the
distraction, should be administered as soon as the problem is recognized.
Peroneal nerve release should be considered if evidence of peroneal nerve
stretch does not respond to slowing distraction.
To minimize the psychologic impact of lengthening, serial
lengthenings and surgical reconstructions should be spaced apart according
to the patient’s age to give the child as much time as possible without
surgery between sessions. We prefer to lengthen when the patient is between
the ages of 1.5 and 4 years for the first lengthening, 6 and 10 years for the
second lengthening, and 12 and 14 years for the final lengthening. We have
found that children between the ages of 4.5 and 6 years have the most
psychologic difficulty with lengthening, whereas children 4 years and
younger have the easiest time with lengthening. We prefer to complete the
last lengthening before the patient is in high school, for social reasons, if
Cost is another argument for reconstruction rather than amputation.
The cost of amputation and prosthetic fitting from age 1 to 18 years is
$81,000 per patient (20). Projected lifetime total costs are $373,051 per
amputee (34). In comparison, the cost of surgical reconstruction is $40,000
to 50,000 for a single surgical lengthening reconstruction (20). Even three
such reconstructions cost less than the lifetime cost per amputee. Therefore,
limb salvage is more cost-effective than amputation.
In our opinion, there are few contraindications to lengthening. We
think that all patients should be given the option of lengthening versus
amputation. If the lengthening option is not available at the treating center,
patients should be offered a second opinion at a referral center that has
expertise in lengthening reconstruction surgery. Socioeconomic factors may
limit such second opinion options. Nevertheless, this should be the patient’s
decision and not the doctor’s. Currently, one of the biggest limitations is the
availability of pediatric orthopaedic surgeons who are trained in advanced
lengthening reconstruction techniques. Finally, in many developing nations,
amputation may be culturally unacceptable and good prosthetics
unobtainable. In such situations, amputation is contraindicated.
Progressive genu valgum after lengthening in patients younger than
12 years is a previously under-recognized problem that can occur as a result
of limb lengthening. Seventy-five per cent of our patients younger than 12
years and essentially all of our patients younger than 4 years developed this
problem. The deformity recurs through the proximal tibia. The origin is
unclear but follows a pattern similar to that seen with the Cozen
phenomenon (11) after proximal tibial metaphyseal fractures. In fibular
hemimelia, the progressive tibial valgus may be related to the lack of lateral
growth by the fibula or may be due to soft tissue tethers on the lateral side.
Intentionally deforming the tibia into 10° to 15° of varus at the end of the
lengthening balances out with the expected rebound leaving the leg well
aligned within the first year. A similar valgus tendency is observed with
progressive valgus deformity in children with fibular hemimelia after
amputation (13). Femoral valgus is also associated with fibular hemimelia
but is nonprogressive (5). Femoral valgus may contribute to valgus overload,
which may be a factor for valgus rebound in the tibia. For this reason we
currently prefer to perform distal femoral hemiepiphsiodesis or varus
osteotomy at the time of the index procedure. Complete fibrous anlage
resection compared to partial anlage resection has reduced the frequency
and degree of rebound but has not eliminated the problem. We prefer to
perform complete resection instead of partial resection.
Some authors have shown growth inhibition after tibial lengthening
for fibular hemimelia (30,31). Sharma et al. (31) concluded that this is
related to complete (type 2) fibular hemimelia. Most of the cases presented
by Sharma et al. (31) were treated with double-level or combined femur and
tibial lengthening without soft tissue release. Hope et al. (19), who used only
single-level lengthening, could not demonstrate any growth inhibition. From
our center Sabharwal et al. (29) showed that growth inhibition occurred only
if there had been a second tibial lengthening performed within a year of the
first. In a larger followup study, Sagieh et al, found evidence of mild
stimulation and mild inhibition in one-third of the cases.
Our classification according to foot pathology is very surgically
oriented. It does not classify the amount of leg length discrepancy. Several
authors include the leg length discrepancy as part of their classification
(7,12,21). We prefer to classify the case according to the hindfoot deformity
and to classify the lengthening according to how many lengthenings will be
required to complete limb length equalization. By combining the foot
classification with this lengthening classification, we have a realistic picture
of how many operations to need to be planned and what type of foot
CONCLUSION
In conclusion, the final result of lengthening for fibular hemimelia is
dependent on the final foot position after reconstruction. It is essential to
obtain a plantigrade stable foot to ensure a satisfactory result. Extra-articular
soft tissue release combined with osteotomy is the best method to obtain a
plantigrade stable foot. Some cases will require ankle fusion to maintain this
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