Leg-Length Discrepancy in Children

Christopher Bradish

Contents Keywords
General Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4345
Aetiology and Classification  Anatomy and
pathology  Complications  Diagnosis  Indi-
Aetiology and Classification . . . . . . . . . . . . . . . . . . . . . . 4345
cations for surgery  Operative technique 
Applied Anatomy and Pathology . . . . . . . . . . . . . . . . 4346 Post-operative care and Rehabilitation  Pre-
Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4348 operative preparation and planning
Indications for Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . 4349
With a Pure Leg Length Discrepancy . . . . . . . . . . . . . 4349
Leg Length Discrepancy Combined with an General Introduction
Angular Deformity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4350
Short Stature . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4350 There are many causes of a leg length discrepancy
Operative Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4351 in children and the treatment of this condition
Tibial Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4353 involves both a thorough clinical and radiological
Femoral Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4354
Congenital Lower Limb Deficiency Syndromes . . 4355
assessment to determine the cause of the defi-
ciency and how it affects the child and
Post-Operative Care and Rehabilitation . . . . . . . . 4368
a prediction of future growth to help decide with
Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4369 regards to the need for intervention. Should inter-
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4369 vention be required, particularly if it involves
limb lengthening, it may be complex and
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4370
time-consuming. In some conditions limb recon-
struction may not feasible and for these children
amputation should be offered at an early age. There
are many treatment options and this subject is one
of the most complex in childrens Orthopaedics.

Aetiology and Classification

The causes of a limb length discrepancy are

summarised below.
Causes of limb length discrepancy:
1. Congenital
C. Bradish
Great Ormond Street Hospital, London, UK (a) Bone dysplasia e.g., Olliers disease,
e-mail: cbradish@blueyonder.co.uk multiple hereditary exostoses

G. Bentley (ed.), European Surgical Orthopaedics and Traumatology, 4345

DOI 10.1007/978-3-642-34746-7_260, # EFORT 2014
4346
(b) Congenital long bone disorder, e.g., prox-
imal focal femoral deficiency, fibular
hemimelia, tibial dysplasia.
(c) Vascular e.g., Klippel Trenaunay Weber
syndrome
(d) Neurological e.g., spinal dysraphism,
sacral agenesis
(e) Neurocutaneous e.g., Neurofibromatosis,
Proteus
(f) Hemihypertrophy syndrome e.g.,
Beckwith Wiedermann
(g) Syndromes associated with asymmetrical
limb growth e.g., Russell Silver,
Rubenstein Taybi,
(h) Idiopathic hemihypertrophy or
hemiatrophy
2. Acquired
(a) Trauma e.g., fracture involving the growth
plate, post radiotherapy or burns
(b) Infection e.g., meningococcal
septicaemia, poliomyelitis, osteomyelitis
(c) Vascular, e.g., femoral artery thrombosis
secondary to cardiac catheterisation
(d) Bone tumour
In some conditions the disorder causes an
overgrowth on the affected side (e.g., neurofibro-
matosis and Klippel Trenaunay), but in others the
involved limb is shorter (e.g., poliomyelitis-with
neurological damage, or post meningococcal
septicaemia-with multiple growth plate arrests,
congenital femoral, tibial or fibular deficiency).
Also to be considered are syndromes which do
not usually cause a limb length discrepancy but
which result in short stature (e.g., achondroplasia,
hypochondroplasia), as these children may also
benefit from limb lengthening.
In assessing the child it is also important to
realise that frequently the problem is not just one
of a limb length discrepancy but that the joints
may also be involved (e.g., the hip in proximal
focal femoral deficiency and the knee with tibial
and fibular deficiency). There may also be an
associated neurological condition (e.g., weakness
with poliomyelitis). In addition not only the
lower limbs but also the spine and upper limbs
may be affected (e.g., with tibial dysplasia and
with VACTERL, that is the association of
Vertebral abnormalities, Anal atresia, Cardiac
C. Bradish
abnormalities, Tracheo-oesophageal fistula,
Renal defect, Radial dysplasia and Lower-limb
abnormalities).
There are classifications for femoral, tibial and
fibular deficiencies which will be described in the
Indications for Surgery section.
Applied Anatomy and Pathology
The molecular regulation of limb growth is
a complex subject, detailed discussion of which
is beyond the scope of this chapter. An excellent
discussion is presented by Lyons and Ezaki [1]
from whom the following summary has been
distilled.
As the signaling pathways for limb differenti-
ation become better understood at a molecular
level, congenital morphological anomalies in
limbs are seen as patterning errors and offer
clues to the role of both genetic and epigenetic
effects.
Investigators now have sophisticated tools of
molecular genetics, such as microarray chips,
which can simultaneously search for abnormali-
ties in the expression of thousands of genes, and
techniques that can snip and splice as well as
amplify and analyze extremely small quantities
of DNA. One of the most revolutionary tools is
the knockout animal, bred specifically to
answer the question What happens if this partic-
ular gene is missing?
With the availability of these molecular
genetic tools, and the compilation of the human
genome, we now have a good, but not perfect,
understanding of what should happen in the nor-
mal processes of limb development.
The limb buds appear in the embryo approxi-
mately 28 days after fertilization along the sym-
metrical lateral mesodermal plates known as the
Wolff crest. The apical ectodermal ridge is
a thickening of the limb ectoderm which forms
at the tip of the limb bud. Skeletal elements
differentiate from proximal to distal with prolif-
eration of cells condensing to form the cartilage
templates of individual bones. The apical ecto-
dermal ridge produces fibroblast growth factors
(FGFs) which promote proliferation and inhibit
Leg-Length Discrepancy in Children
the condensation and differentiation in mesen-
chymal cells nearest the apical ectodermal
ridge. As the limb grows outward, the proximal
mesenchymal cells that are farthest from the api-
cal ectodermal ridge and destined to become
skeletal elements undergo condensation and ini-
tiate chondrogenesis.
The development of this proximal-distal axis
is integrated with the development of the
anterior-posterior axis (in human development
this corresponds to the radial-ulnar axis in the
upper limb, and tibial-fibular axis in the lower
limb). This axis is controlled by the zone of
polarizing activity, a region of limb mesenchyme
located on the posterior side of the limb bud near
its junction with the flank mesenchyme. The zone
of polarizing activity is the source of the secreted
protein Sonic hedgehog (Shh) which controls the
radial-ulnar or tibial-fibular patterning of limbs
and specifies both digit number and digit identity
Beyond its role in specifying digit number and
identity, Shh is required for proximal-distal out-
growth. The requirement for Shh in regulating
outgrowth is seen in Shh-deficient mice, in
which the limbs are truncated at the most proxi-
mal skeletal element. This aspect of Shh function
is a reflection of the reciprocal requirement for
Shh to maintain the expression of FGFs in the
apical ectodermal ridge. Thus, loss of either Shh
or FGFs in the limb bud leads to limb truncation
and therefore loss of differentiation distally.
Bone Morphogenic Proteins (BMPs) and Grem-
lin, a secreted inhibitor of BMPs, also participate
in this pathway; the BMPs produced throughout
the limb mesenchyme and in the apical ectoder-
mal ridge inhibit production of FGFs by the api-
cal ectodermal ridge. A key role of Shh is to
activate expression of the BMP antagonist Grem-
lin, thereby preventing this inhibition of FGF
expression. Hence, overexpression or
underexpression of FGFs, Shh, or BMPs in the
early limb bud leads to phenotypes associated
with limb truncations or patterning defects (alter-
ations in digit identity and number), but not to
limb overgrowth.
The earliest overt sign of chondrogenesis is
the aggregation of limb-bud mesenchymal cells
into precartilage condensations, the sizes and
4347
shapes of which are specified by the actions of
the Shh and FGF gradients. The exact mechanism
of chondrogenesis is almost entirely unknown;
however, members of the Hox family of tran-
scription factors are involved in this process.
Hox genes are expressed in nested patterns
along the proximal-distal axis of the limb bud,
and mutations of these genes lead to alterations in
the sizes and shapes of precartilage condensa-
tions in mice and humans. The mechanism by
which Hox genes control growth are largely
unknown, however recent studies have shown
that they directly regulate the expression
of BMPs.
The formation of the growth plate occurs with
the proliferation of chondrocytes which is under
the control of BMPs and transcription factor. The
proliferation of chondrocytes in the growth plate
is under the regulation of secreted Indian Hedge-
hog and Parathyroid Hormone Related Protein.
Unfortunately knowledge about growth regu-
lation has not directly transferred to help
elucidate clinical conditions. For example,
germline mutations in genes that regulate FGF,
BMP, Shh, and PTHrP pathways have not been
shown to be associated with generalized or wide-
spread disproportionate growth of limb segments
in hemihypertrophy conditions or in the
hamartomatous overgrowth characteristic of Pro-
teus and Proteus-like syndromes.
In contrast to the large amount of informa-
tion regarding the molecular details of limb
growth, essentially nothing is known about the
mechanisms underlying the cessation of limb
growth. However, there is new evidence that
negative-feedback pathways involving BMPs,
FGFs, and Shh supply the signals to cease
limb outgrowth.
Acquired conditions are easier to understand
and largely result from damage to the growth
plate causing a premature growth arrest that will
result in a limb shortening if the arrest is central,
and an angular deformity if there is a peripheral
arrest. In addition if there is neurological damage
with limb weakness this will inhibit the growth
of the limb and is responsible for the shortening
seen with, for example, poliomyelitis or spinal
dysraphism.
4348
Diagnosis
A limb length disorder may be diagnosed in utero
as a result of an ultrasound scan. A detailed scan
can reveal not only a discrepancy in length but also
define the status of the long bones and the foot and
enable the diagnosis of for example proximal focal
femoral deficiency (PFFD). In the majority of
cases however the diagnosis is made postnatally.
The diagnosis then involves assessment not only
of the limb but of the whole child to determine
whether there are any associated abnormalities,
e.g., ulnar hemimelia with a tibial dysplasia,
VACTERL. The limb is examined to determine
whether the discrepancy is predominantly femoral
(as with PFFD) or below the knee (with tibial and
fibular dysplasias). The joints are examined as the
hip is deficient with PFFD and the knee is fre-
quently involved with all the lower limb dyspla-
sias (with deficient anterior and posterior cruciate
ligaments). The fibula is partially or completely
absent in fibular hemimelia. The foot may be
abnormal, with absent lateral rays with fibular
hemimelia and cleft or augmented with tibial dys-
plasia. A tarsal coalition, with the talus and calca-
neum being a single bony, is frequently seen with
fibular hemimelia. The lower limbs are examined
not only for length but also to determine if there is
any angular or rotational malalignment.
When assessing the child with short stature it is
important to assess whether there is proportionate
short stature (i.e., the child is in proportion but is
below the third centile for height as with, for exam-
ple, intrauterine growth retardation and spondyloe-
piphyseal dusplasia) or there is a greater shortening
of the limbs (as with for example achondroplasia).
The actual leg length discrepancy can be deter-
mined by measurement or by asking the older
child to stand on graded blocks to level the pelvis.
The child should be observed both standing and
walking to assess the gait and any compensatory
deformities, e.g., an equinus contracture to com-
pensate for a short leg or subtalar inversion to
compensate for a tibial valgus deformity.
The associated features which are sought are:
1. Cutaneous e.g., cafe au lait pigmentation with
neurofibromatosis
C. Bradish
2. Vascular e.g., increased vascular markings
with Klippel Trenaunay syndrome
3. Soft tissue e.g., soft tissue overgrowth with the
proteus syndrome and neurofibromatosis
4. Bony abnormalities such as multiple exostoses
5. Spinal examination to exclude spinal
dysraphism with a spina bifida defect or cuta-
neous abnormality such as an area of increased
hair growth. Also to detect a scoliosis with for
example neurofibromatosis.
6. Upper limb examination to detect e.g., ulnar
hemimelia and radial hemimelia.
7. Facial examination to detect syndromes such
as Russell Silver, Beckwith, Rubinstein Taybi,
achondroplasia.
8. Neurological examination to exclude weak-
ness with, for example, polio or spinal
dysraphism.
The further investigations which are required
include:
Long leg X-rays (both anteroposterior and
lateral) to determine the limb length difference
and to perform the malalignment test which
enables determination of the limb axis and any
deviation from normal [2].
Xray pelvis to determine status of hips. This
is important with femoral deficiencies as hip
reconstruction may need to precede limb recon-
struction. Also in the presence of hip dysplasia
the hip is at risk of subluxation/dislocation with
femoral lengthening and an acetabuloplasty
should be performed prior to femoral lengthening
if the centre- edge angle is <20 .
Xrays femur, tibia/fibula to define any defi-
ciency and to enable grading of the deformity.
The status of the growth plates needs to be
established in meningococcal septicaemia and
where the shortening is due to trauma or infection
with physeal damage.
Additional investigations include:
MRI scan of hip (in PFFD) and of knee/tibia (in
tibial dysplasia), again to define anatomy and
aid grading and surgical planning.
Skeletal survey to aid diagnosis in bone dysplasias.
Abdominal ultrasound with Beckwith syndrome
to exclude the association of Wilms tumour.
For syndromic deformities the geneticist can
help with diagnosis and prognosis and advise
Leg-Length Discrepancy in Children
the family with regard to the risk of recurrence in
future children.
Having established the cause of the limb
abnormality one tries to predict what will
occur with skeletal growth and the magnitude
of the deformity at skeletal maturity. Patterns
of growth with skeletal deformities were
defined by Shapiro [3] who described five pat-
terns of progression of leg length discrepancy
in children. In type 1 there is a proportionate
increase in the discrepancy with growth, this
pattern is seen in most congenital and
developmental causes of LLD. For this reason
the LLD at skeletal maturity can be predicted.
Types 25 have periods of acceleration or
deceleration and cannot be predicted accu-
rately. (Type 2 is seen with neuromuscular dis-
ease and some congenital hypo or hyperplasias,
type 3 with a brief acceleration of growth is
seen typically following a fracture, type 4
with a brief cessation of growth is seen with
for example Perthes where there is collapse of
the femoral head followed by premature
physeal arrest, type 5 with acceleration of
growth then premature growth arrest is seen
with inflammatory arthritis).
The discrepancy at maturity in Shapiro Type 1
can be predicted by various methods including
the Paley Multiplier, Moseley straight line graph
and growth remaining (Fig. 1). All of these
methods depend on the data of Anderson and
Green from 1964 where Xrays were used to deter-
mine the femoral and tibial lengths in children
from age 5 to skeletal maturity.
The easiest method to use is that of Paley who
has determined multipliers to be used at any
given age. If the leg length difference at a given
age is multiplied by the appropriate multiplier
this will give the predicted LLD at skeletal
maturity [4].
Indications for Surgery
The aim of any surgical intervention is to maxi-
mise function. In addition if undertaking stature
lengthening there are also cosmetic consider-
ations but these should always be secondary.
4349
DISCREPANCY
TYPE 1 UPWARD SLOPE PATTERN
AGE
TYPE 2 UPWARD SLOPE-
DECELERATION PATTERN
TYPE 3 UPWARD SLOPE-
PLATEAU PATTERN
TYPE 3A DOWNWARDD SLOPE-
PLATEAU PATTERN
TYPE 3B PLATEAU PATTERN
TYPE 4 UPWARD SLOPE-PLATEAU-
UPWARD SLOPE-PATTERN
TYPE 5 UPWARD SLOPE-PLATEAU-
DOWNWARD SLOPE PATTERN
Fig. 1 Shapiro leg length discrepancy types (From Sha-
piro F.J.Bone Joint Surg(Am)1982.64;639-5. By kind
permission of the J.Bone and Joint Surgery)
There are various indication for surgery:
A. With a pure leg length discrepancy
B. Leg length discrepancy combined with an
angular deformity
C. Short stature.
With a Pure Leg Length Discrepancy
In an adult leg length differences up to 2 cm are
well tolerated and do not require treatment. With
a predicted discrepancy in excess of 2 cm there
are the following options:
1. A shoe raise. This is suitable for limb discrep-
ancies up to approximately 4 cm, however
many families reject the concept of a shoe
raise and wish for a definitive solution.
2. Epiphyseodesis to slow the growth of the lon-
ger leg. This can be femoral alone, tibial alone
or combined, depending on the magnitude of
the deformity.
3. Surgical shortening of the longer leg. It is
possible to shorten a limb segment by up to
4350
7.5 cm in the femur and 5 cm in the tibia in
adult males [5]. This can be undertaken as an
open technique with either blade plate or
intramedullary fixation. Alternatively a closed
technique using an intramedullary saw to
shorten the limb by the required amount and
then an intramedullary nail for stabilisation
can be used [6].
4. Surgical lengthening of the short leg. This
can be undertaken gradually using an exter-
nal fixator (monolateral or circular frame) or,
when skeletally mature, by using an extend-
able intramedullary nail (e.g., ISKD, Precise,
FitBone or Albizzia). A lengthening up to
5 cm is readily achievable at one treatment
session, for larger discrepancies the treatment
may need to be phased with repeated
lengthening.
5. With a predicted discrepancy in excess of
1015 cm these treatments may need to be
combined with for example a lengthening of
the shorter leg plus an epiphyseodesis of the
longer leg.
6. With a discrepancy greater than 1520 cm, par-
ticularly if the child has a poorly developed foot,
an amputation is frequently the best treatment in
terms of maximising function. The child will
then be provided with a suitable prosthesis to
equalise the leg lengths.
7. A Van Ness rotation plasty, with rotation of the
lower limb through 180 (and shortening if
required) in order to place the ankle at the level
of the normal opposite knee, with the arc of
movement of the ankle mimicking that of the
knee so that the dorsiflexed ankle gives a flexed
knee and the plantarflexed ankle gives
a straight knee. This then enables fitting of
a prosthesis with the ankle joint providing active
knee flexion and extension.
Leg Length Discrepancy Combined
with an Angular Deformity
Many conditions cause a combined deformity, e.g.,
Olliers disease, multiple hereditary exostoses, post
meningococcal septicaemia. Any angular (and
rotational) deformity should be corrected in order
C. Bradish
to minimise excessive joint reaction forces and to
delay the onset of degenerative arthritis. The
malalignment test detailed above enables any
angular deformity to be measured and the site of
the deformity (the Centre of Rotation of Angula-
tion or CORA) to be defined. There are the follow-
ing options for correcting an angular deformity:
1. If the CORA is at the growth plate, or close to
the growth plate, and the growth plate has nor-
mal growth potential, a hemiepiphyseodesis is
appropriate.
2. If the CORA is meta-diaphyseal or diaphyseal
then an osteotomy and either acute correction
(an opening or closing wedge) or gradual
frame assisted correction is appropriate.
Obviously if there is an associated limb
length discrepancy a lengthening can be
undertaken at the same time if using an exter-
nal fixator.
3. If there is extensive growth plate involvement,
such that any future growth will just cause
a recurrent angular deformity, as is seen post
meningococcal septicaemia, then the growth
plate should be ablated and angular correction
and lengthening undertaken. With a joint
deformity, such as is seen in Blounts disease,
a hemiplateau elevation can be combined with
ablation of the growth plate, angular correc-
tion and lengthening.
Short Stature
Stature lengthening is a possibility but it remains
controversial. For children with syndromic short
stature (e.g., achondroplasia) many families
believe that it is unnecessary to intervene where
the child is otherwise fully active and contented,
lengthening will achieve height but will not erad-
icate the condition and make the child normal.
Other families and children believe that signifi-
cant short stature is both a cosmetic and a social
handicap and that as the technology exists to
improve the situation the opportunity should not
be missed.
There is also the situation with constitutional
short stature where there is no identifiable syndrome
but the person is short and requests lengthening.
Leg-Length Discrepancy in Children
This is not normally offered in the UK but is cer-
tainly undertaken in some countries [7, 8].
The child or young adult will require
a thorough assessment to determine the cause
of the short stature and to define the limb
deformities. Lengthening combined with
angular correction may be required. Counselling
will also be required to be certain that lengthen-
ing is the correct treatment and that there are
not significant psychological problems, this is
particularly important with constitutional short
stature.
For stature lengthening in achondroplasia
crossed lengthening is usually undertaken, length-
ening one femur and the contralateral tibia. When
the child has recovered from this surgery the oppo-
site femur and tibia are similarly lengthened. This
strategy ensures that a limb length discrepancy is
not created. A 58 cm increase in height can readily
be achieved with each lengthening and the process
can be repeated to achieve at least a 2025 cm
increase. The process can be commenced when
the child is 56 years old, with further lengthenings
at intervals of 12 years, tailored to fit in with the
childs education. For constitutional short stature
Catagni et al. [7] reported bifocal bilateral tibial
lengthening with an Ilizarov fixator, gaining
a mean of 7 cm in height. An alternative is to use
an intramedullary lengthening device as this over-
comes the potential problems of prolonged external
fixator treatment and causes less scarring.
Operative Technique
In order to lengthen a limb the bone needs to be
divided and then gradually distracted while new
bone forms in the distraction gap, a technique
known as distraction osteogenesis. This new
bone will then gradually consolidate to normal
healthy bone. The distraction can be undertaken
using an external fixator (monolateral or circular
frame), or an intramedullary distraction device.
Intramedullary distraction is usually reserved for
around the time of skeletal maturity because of
the concern that intramedullary nailing may
cause epiphyseal damage or, particularly, femo-
ral head avascular necrosis. In addition the
4351
medullary canal needs to be of sufficient size to
accommodate the intramedullary nail.
The technique of bone division and subse-
quent lengthening is crucial to success. Distrac-
tion osteogenesis was developed by Ilizarov in
Russia and he described a corticotomy for bone
division, whereby the cortex of the bone alone is
divided, having approached the bone
subperiostally and being careful not to divide
the intramedullary blood vessels. He believed
that it was important to preserve the
intramedullary blood supply for bone regenera-
tion. In addition he stressed that there should be
a delay between the corticotomy and commenc-
ing distraction so that new bone formation could
commence and that this new bone column could
then be lengthened, a process subsequently called
callotasis (literally callus distraction).
Subsequently it has been realised that preser-
vation of the medullary vessels is not crucial as
these will reform during the period of delay prior
to lengthening. However the term corticotomy
has become part of the lengthening vocabulary.
The crucial steps in any limb lengthening
procedure are:
1. The procedure is undertaken under a general
anaesthetic. No tourniquet is used and the
patient is not paralysed. This is particularly
important if using a fine wire circular frame
external fixator so that should a wire inadver-
tently be passed through a vessel or nerve
there will be feedback of either bleeding or
a muscle twitch, indicating that the wire
should be removed and reinserted in a safe
corridor.
2. The bone division should be in the metaphysis
if possible as much better bone formation is
achieved here than in the diaphysis. The femur
can be lengthened either in the proximal or
distal metaphysis. Fewer knee problems are
seen with a proximal femoral lengthening,
particularly when treating the congenital
short femur [9]. For tibial lengthening the
fibula also requires division, this can be safely
preformed at the junction of the upper 2/3,
lower 1/3. Tibial lengthening is usually under-
taken at the proximal metaphyseal level.
A bifocal lengthening can be undertaken with
4352
simultaneous proximal and distal lengthening
but this is rarely indicated in children unless
simultaneous angular deformity correction is
being undertaken at both levels.
3. The bone should be approached subperiostally
and with minimal periosteal stripping as the
periosteum is crucial for new bone formation.
The periosteum is incised longitudinally over
a distance of approximately 23 cm and retrac-
tors placed beneath the periosteum to protect it
while bone division is being performed.
4. A low energy bone division with either a drill
and osteotome or a Gigli saw is undertaken to
minimise thermal damage which can delay
bone regeneration.
5. There is a delay of 57 days between the
corticotomy and commencement of distraction.
6. Lengthening is undertaken at a rate of approx-
imately 1 mm per day. If lengthening is under-
taken too quickly there will be poor regenerate
formation. Conversely if undertaken too
slowly premature consolidation may occur
with healing of the bone before the desired
lengthening has been achieved. A repeat
corticotomy would then be required. In the
tibia a proximal corticotomy can be length-
ened at 1 mm daily but a distal corticotomy
is usually lengthened at 0.75 mm daily
because of slower bone formation distally.
Lengthening is continued until the desired
length has been achieved. During this period
Xrays of the regenerate are undertaken
2 weeks post operatively and then at approxi-
mately monthly intervals to ensure that the
regenerate column is satisfactory. Normally
new bone is visible after 2 weeks and one
wishes to see a regenerate column which is
the same width as the normal bone above and
below (normotrophic). If the regenerate is
narrower (hypotrophic) this suggests that
bone formation is slow, either because of too
fast a distraction rate or because of poor local
biology (e.g., previous periosteal damage,
poor coticotomy technique or with some con-
ditions such as Turners syndrome). The rate
of distraction then needs to be slowed down to
0.75 or 0.5 mm daily (oestrogen supplements
C. Bradish
may help in Turners). If the bone column
is wider than the bone above and below
(hypertrophic) this indicates too rapid regen-
erate formation and the distraction rate
needs to be increased to prevent premature
consolidation. Hypertrophic regenerate is typ-
ically seen with achondroplasia and Olliers
disease and in these conditions distraction
may need to be at 1.251.5 mm daily for the
first 2 weeks.
7. Throughout the period of treatment the patient
is encouraged to weight bear as much as tol-
erated and receives regular physiotherapy
(at least twice weekly) to try and maintain
the joint range of motion. When undertaking
femoral lengthening a knee flexion contrac-
ture typically develops and the physiotherapist
endeavours to maintain full extension and
flexion to at least 45 . If a fixed flexion defor-
mity develops this will be difficult to over-
come following completion of lengthening.
In addition if the knee is unstable with cruciate
deficiency the flexed knee is likely to sublux
posteriorly. Similarly during tibial lengthen-
ing an ankle equinus contracture typically
develops. If the child has been walking on
tip-toe prior to surgery to compensate for
a leg length discrepancy there may be
a reduced range of ankle dorsiflexion. Physio-
therapy can usually maintain a plantigrade
foot when undertaking a lengthening up to
5 cm, provided there is a normal range of
ankle dorsiflexion prior to treatment. With
a reduced ankle dorsiflexion range prior to
surgery (to 10 above neutral only) the hind
foot should be included in the frame to main-
tain the foot plantigrade while lengthening.
With a greater reduction a percutaneous
Achilles tendon lengthening or gastrocnemius
slide should be undertaken to increase the
ankle dorsiflexion range at the time of appli-
cation of the fixator and then similarly the hind
foot should be included in the frame. As soon
as the desired lengthening has been achieved
the foot frame can be removed to permit ankle
exercises, while the remainder of the frame
remains in place until bone consolidation.
Leg-Length Discrepancy in Children
8. The fixator needs to remain in place until the
regenerate new bone has consolidated suffi-
ciently to enable its removal without risk of
subsequent fracture or bowing deformity.
This is always a difficult decision. The regen-
erate initially has a uniform appearance on
Xray but then gradually differentiates to form
a cortex and a medullary canal. A normal
bone will show two cortices on both an
antero-posterior and a lateral X-ray, giving
four cortices in total. The regenerate is suffi-
ciently strong for fixator removal when a total
of three cortices can be seen on the AP plus
the lateral X-ray.
If distraction is undertaken at a rate of
1 mm daily, a 1 cm lengthening will be
achieved in 10 days. The consolidation period
in children is usually double the distraction
period and so a 1 cm lengthening would
require 20 days of consolidation, giving
a fixator index (total fixator time in days
divided by gain in length in cm) of a month
per cm.
Because of the prolonged fixator time to
achieve consolidation efforts have been
made to reduce the fixator time by either
lengthening over a nail (LON) which can
be locked at the completion of lengthening
and the fixator then removed, or by
stabilising the bone by a locking plate at
completion of lengthening [10]. This can
reduce the fixator index to approximately
15 days per cm.
9. When the bone has consolidated the fixator is
removed. This is usually done under general
anaesthetic. Continued protected weight bear-
ing is advised until complete radiological
healing is visible on Xray.
The techniques relating to specific pathologies
will now be discussed.
Tibial Lengthening
For a congenital short tibia, in the absence of
fibular hemimelia and where lengthening alone
is required, the steps are as follows:
4353
The fibula is first divided at the junction upper
2/3, lower 1/3. Division can be with an
oscillating saw as a low energy bone division
is less critical in the tibia. The wound is
then closed.
The fixator is then applied. Whether
a monolateral or circular frame is used is not
critical, both are well tolerated and equally
satisfactory results can be achieved with
either. The circular frame is more versatile
and if undertaking complex deformity correc-
tion familiarity with circular frames is essen-
tial. For this reason many surgeons will use
circular frames for the majority of lengthening
procedures. If using a circular frame either
a conventional Ilizarov fixator can be used
with the lengthening being performed by
lengthening four circumferential rods, or the
Taylor Spatial frame can be used with the
lengthening being achieved by lengthening
the six struts. The Spatial frame has the advan-
tage that should a deformity develop during
lengthening then it can be corrected by
adjusting the struts, whereas a conventional
Ilizarov would require modification of the
frame by insertion of hinges (for application
of tibial Spatial frame see [11]).
With a circular frame for tibial lengthening
two rings only are required, the proximal
secured by wires or half pins just distal to the
proximal physis and the distal approximately
2 cm above the distal physis. A wire needs to
be placed from fibula to tibia both proximally
and distally to ensure that the fibula lengthens
along with the tibia. If this is not done the
proximal fibula may migrate distally during
lengthening, stretching the biceps femoris
and generating a knee flexion contracture.
Similarly the distal fibula may migrate proxi-
mally, disrupting the ankle mortice and risking
an ankle valgus deformity. The peroneal nerve
is at risk from the proximal fibular wire and
a peroneal nerve decompression can be
preformed prior to inserting this wire and the
nerve protected during the wire insertion. If
applying an all wire frame it is important that
when inserting the distal wires the ankle is
4354
plantarflexed when going through the anterior
compartment and dorsiflexed when going
through the posterior compartment so that the
ankle excursion is not restricted.
Having applied the fixator the corticotomy is
then performed, 1 cm distal to the most distal
of the proximal wires or half pins. This
ensures that the proximal fixation is not
jeapordised and that the corticotomy is
metaphyseal. The image intensifier can be
used to check that the corticotomy is com-
plete. Alternatively the proximal and distal
rings can be disconnected and the distal por-
tion of the tibia externally rotated to be cer-
tain of its completion (it is not internally
rotated as this would risk stretching the pero-
neal nerve). The wound is then closed.
In order to try and minimise the risk of pin or
wire infection careful attention to detail is
essential, both when inserting the wires/pins
and subsequently. The regime as described by
Nayagam and his co-workers from Liverpool
(based on that used in Russia) is advocated
[12]. This involves the following steps:
Non-touch handling of wires/pins
Pulsed drilling (stop-start) with irrigation
Bone swarf removal
Immediate pin-site dressing in alcoholic
solution of chlorhexidine with pressure to
reduce haematoma; changed at completion
of the operation if bloodstained.
Pin sites cleaned daily for 3 days to ensure
blood removal; pin cleaning using
a solution of 70 % alcohol and dressings
moistened with chlorhexidine.
Occlusive pressure dressing after the third
day; pin cleaning and dressing changes
repeated every 710 days using the same
technique.
The patient is encouraged by the physiother-
apists to commence weight bearing on the
day following surgery and to progress
to walking with a frame or crutches by the
time of discharge at approximately 57 days
following surgery. By the same time the
carers will have been instructed in care of
the fixator and will have been taught how to
undertake the lengthening.
C. Bradish
Femoral Lengthening
For a simple femoral lengthening (i.e., with
a femoral growth arrest and where there is no
angular deformity) the steps are as follows:
If using a circular frame external fixator the
distal ring is applied first. If using a conventional
Ilizarov with an all wire frame it is best to insert
the four distal wires prior to application of the
ring so it can be ensured that the wires are not
interfering with knee flexion and extension. Two
transverse wires with counterposed olives are
first inserted, parallel to the knee joint and sepa-
rated by approximately 1 cm. The wires should
not come below the superior margin of the patella
so as to remain clear of the synovial cavity of the
knee. The knee is flexed and extended with each
wire to be sure that they are neutral wires in the
intermuscular plane (vastus lateralis in fact
comes below the mid line so the lateral to medial
wire needs to be inserted with the knee flexed
when entering on the lateral side). A neutral wire
should not bend significantly with knee move-
ment. Two oblique wires are then inserted at
approximately 45 to the horizontal, going
through the anterior compartment with the knee
flexed and exiting the posterior compartment
with the knee in extension. Again these wires
should show minimal bending with knee move-
ment. When four satisfactory wires have been
inserted, with a longitudinal separation of
approximately 2 cm, the ring is secured to the
wires and they are tensioned. It is useful to tem-
porarily place a 1520 cm rod on the ring anteri-
orly to use as a reference to check the alignment
of the ring to ensure that it is not being secured in
flexion or extension, this rod should lie parallel to
the axis of the thigh and in line with the mechan-
ical axis of the leg. The same principles apply if
using a Spatial frame but here the ring would be
secured with a combination of wires and
half pins.
Having secured the distal ring the frame is
then built up with a proximal arch or 5/8 ring.
The proximal and distal arch/ring are
then connected with appropriate rods or struts.
The corticotomy is usually performed distally,
1 cm above the most proximal of the distal
Leg-Length Discrepancy in Children
wires or half pins. A 12 cm lateral skin incision
is made and a 1 cm osteotome used to deepen this
down to the periosteum of the lateral femur. The
osteotome is used to incise the periosteum and to
elevate it anteriorly and posteriorly. Multiple drill
holes are then made at the site of the corticotomy
with a 3 mm drill protected by a drill sleeve. The
osteotome is then used to complete the
corticotomy. If an Ilizarov osteotome with
a hexagonal handle is used a spanner can be
passed over the handle and completion of the
corticotomy checked by rotation of the osteotome
through 90 .
As with tibial lengthening, distraction is then
commenced at 1 mm daily at 5 days.
Congenital Lower Limb Deficiency
Syndromes
Congenital short femur, fibular hemimelia and
tibial dysplasia have a significant overlap.
A child with a congenital short femur for
instance may frequently have fibular hemimelia
affecting the same leg. There is also significant
variability with regards to severity and predicted
limb shortening. It is useful however to separate
these conditions in terms of management. With
the severe form of congenital short femur with
proximal focal femoral deficiency the treatment
will be largely determined by the condition of
the hip. With a tibial dysplasia the treatment will
be determined by the condition of the knee and
ankle. If the predominant deformity is fibular
hemimelia the treatment is determined by
the predicted shortening and the condition of
the foot.
Congenital Femoral Deficiency
The child may be born with significant shortening
of the femur and this may be associated with
failure of normal development of the hip joint,
this is then called Proximal Focal Femoral Defi-
ciency (PFFD). It is more useful to consider
a spectrum of abnormality under the umbrella
heading of congenital femoral deficiency (CFD).
CFD is a rare condition with a quoted inci-
dence of 1 in 50200,000 [13]. The aetiology is
4355
unknown. Two hypotheses have been advanced,
the theory of sclerotome subtraction proposes
injury to neural crest cells that are the precursors
to sensory nerves at the level of L4 and L5.
Histological studies of a fetus with unilateral
PFFD have prompted an alternative hypothesis
that PFFD is caused by a defect in maturation of
chondrocytes (cartilage cells) at the growth plate.
In either hypothesis, the agent causing the injury
is usually not known. Thalidomide is known to
cause PFFD when the mother is exposed to it in
the fifth or sixth week of pregnancy, and it is
speculated that exposure to other toxins during
pregnancy may also be a cause. PFFD occurs
sporadically, and does not appear to be heredi-
tary. There is a rare syndrome, proximal focal
deficiency unusual facies syndrome, where CFD
is seen in a child with a short broad nose and there
may be a cleft palate. Females are more
commonly affected than males, most cases are
sporadic.
Paley has described three types of congenital
femoral deficiency (Fig. 2) and this classification
is the most useful when considering treatment for
this condition [14]:
Type 1 There is an intact femur. The hip and
knee are present and there is continuity between
femoral head and shaft. There is normal ossifica-
tion in type 1A and delayed ossification in
type 1B.
Type 2 There is a mobile pseudarthrosis
between the greater trochanter and the femoral
head. The femoral head is mobile in Type 2A, in
Type 2B the femoral head is fused or absent.
Type 3 There is a diaphyseal deficiency. The
upper end of the femur is absent. The knee has
>45 movement in Type A and is stiff with <45
in type B.
Management Type 1A
With a type 1A with normal ossification of
the femoral neck the femur will be short
with an external rotation deformity, a varus
femoral neck and a valgus knee. These defor-
mities can be corrected by bifocal femoral
treatment, correcting varus and rotation via
a proximal corticotomy and undertaking
lengthening and correction of valgus via
4356
Fig. 2 The Paley
classification of congenital
femoral deficiency
(With kind permission of
Dror Paley)
a. Normal Ossification
Type 2
Mobile Pseudarthrosis
a. Mobile: Femoral Head
and Knee
a distal corticotomy. It is important that the
fixator be extended to the knee, with a hinge at
the level of the knee, to prevent subluxation
of the knee during lengthening. Treatment
can be undertaken with an Ilizarov fixator or
monolateral fixator.
An alternative for the less severe deformity is
monofocal treatment with an Ilizarov fixator. In
order to prevent problems with knee flexion con-
tractures and femoral deformity following
lengthening, lengthening via a proximal femoral
corticotomy over a pre placed Rush pin is
recommended [8].
It is important to undertake soft tissue releases
with CFD, the soft tissues are abnormal and
contracted. The iliotibial band needs to be
excised from the hip to the knee and the rectus
femoris tendon lengthened at the hip. The
biceps and medial hamstrings may also need
lengthening. Botox can be injected to the
quadriceps muscle to reduce spasm and to aid
postoperative physiotherapy.
If the centre edge angle of the hip is less than
20 the hip will be unstable and prone to sublux-
ation during lengthening and so a preliminary
pelvic osteotomy (Dega) should be undertaken
prior to lengthening.
C. Bradish
PALEY CLASSIFICATION
Type 1
Intact Femur, Mobile Hip and Knee
b. Delayed Ossification
Type 3
Diaphyseal Deficiency of Femur
b. Femoral Head Stiff or Absent a. Knee Partially Mobile b. Knee Stiff
Mobile Knee > 45 < 45
Management Type 1B
With delayed ossification there will be
a significant deformity to the proximal femur.
The proximal femur may be bent back on itself
into varus with flexion and abduction and exter-
nal rotation contractures of the hip. In order to
address this Paley has developed the Super
Hip operation. This is a complex procedure
but with it is possible to address all aspects of
the deformity. Extra articular soft tissue
releases are performed to correct the flexion
and abduction contractures, a subtrochanteric
osteotomy is performed to correct varus, flex-
ion and external rotation deformities and
a Dega osteotomy is performed to address ace-
tabular dysplasia.
Via a lateral incision and an anterior knee
incision the fascia lata is excised. The tensor
fascia lata muscle is preserved. The fascial lata
is left attached distally for later knee reconstruc-
tion. The tendons of rectus femoris, psoas and
piriformis are divided. The leg is then fully
adducted and if sufficient soft tissue release has
been undertaken the tip of the greater trochanter
should come down to the level of the centre of
the femoral head. This is determined with an
image intensifier. If there is an abduction
Leg-Length Discrepancy in Children
85 45
50
130
Bone
Cartilage
Fig. 3 Pre-operative planning (With kind permission of
the Journal of Bone and Joint Surgery[Am])
contracture the tendons of gluteus medius and
minimus need to be elevated from the greater
trochanter.
Next the bony deformity is addressed. An on
table arthrogram is performed to check that the hip
can be brought to a neutral position. The
arthrogram also aids insertion of a guide wire up
the unossified femoral neck to the centre of the
femoral head. A wire is placed from the tip of the
greater trochanter to the centre of the femoral head
and then a second wire is placed at a 45 angle to
this, passing up the unossified femoral neck, as
shown in the diagram (Figs. 3, 4a, b). This angle
ensures that the 130 blade plate which will sub-
sequently be inserted is in the correct position.
A cannulated chisel is then inserted over the
guide wire and a 130 blade plate inserted. This
will lie away from the shaft because of the signif-
icant deformity requiring correction.
Once the plate is in place the correction can be
determined. A notch is cut in the femur, just distal
to the entry point of the blade plate, equal to the
width of the femur and a second cut at an angle to
the first cut as shown. The femur is internally
rotated to correct the external rotation deformity
and the femur realigned as shown. Some femoral
shortening is essential to reduce the soft tissue
tension. The bone segment removed can be used
for the subsequent Dega osteotomy (Fig. 4b).
Screws are inserted to secure the plate.
A development proposed by Paley is then to
insert BMP-2 up a drill hole in the femoral neck
in order to encourage ossification (Fig. 4c). This is
4357
not currently an approved use of this substance
but he reports that it significantly enhances the
ossification.
A Dega osteotomy is then performed, utilising
the previously removed segment of femur to
maintain the correction. In addition approxi-
mately 1 cm of the iliac crest is resected to take
the tension off the abductors (Fig. 4d, e).
The soft tissues are then repaired by re-
suturing the glutei, rectus femoris and tensor fas-
cia lata to the greater trochanter.
The following Xrays show the pre and post
operative appearances of one of Paleys patients
and the significant ossification of the femoral neck
which is seen 3 months later. The screw in the tibia
is from a subsequent cruciate ligament reconstruc-
tion, the Super Knee, undertaken utilising the
facia lata. This is split and half is passed (Fig. 5)
from posterior to anterior, passing deep to the
lateral collateral ligament and then around the
back of the lateral femoral condyle before exiting
through a tibial drill hole to recreate an anterior
cruciate ligament. The other half is looped around
adductor magnus to create an extra-articular pos-
terior cruciate ligament (Fig. 6).
The extra-articular posterior cruciate prevents
the knee from externally rotating.
Management Type 2 A: Mobile
Pseudarthrosis
In type 2 there is no connection between the
upper femur and femoral head. There is
a greater trochanter in 2A and a mobile femoral
head. The femoral head does not move in type
2B. It is possible to reconstruct the proximal
femur in 2A but this is much more complicated.
The diagram illustrates how the glutei are
detached from the greater trochanter, the psoas
is lengthened and then osteotomies are performed
to recreate a femoral neck (Super Hip 2 proce-
dure) (Fig. 7ad).
Having done these cuts the trochanteric frag-
ment can be rotated through 135 and brought to
the femoral head and secured with threaded
wires.
Further stability is provided with a Rush pin
and tension band. The muscles are then
reattached. Even with this the fixation is tenuous
4358 C. Bradish

a b

Cannulated blade
plate inserted over
guide wire

With osteotomy completed,

distal segment reduced to
proximal segment

d
Osteotomy levered
distally to cover
femoral head.

Channel packe
with Bone
Morphogenic
Protein (BMP)

GM

Gluteus medius
and vastus
lateralismuscles
sutured to VL
greater trochanter

RF

Fig. 4 (a) Insertion of blade-plate. (b) Femoral muscles (With kind permission of the Journal of Bone and
osteotomy and re-alignment. (c) Insertion of B.M. protein. Joint Surgery[Am])
(d) Pelvic covering osteotomy. (e) Reconstitution of glutei
Leg-Length Discrepancy in Children 4359

Fig. 5 X-rays showing

correction, fixation and
fusion. The appearance 3
months following a Super
Hip procedure with BMP-
2 inserted up the femoral
neck

and Paley recommends that a spanning fixator be having achieved a stable hip and knee, limb
applied from pelvis to femur to knee. lengthening surgery can be commenced at around
Both Super Hip operations can be under- age 3 years. The child may require at least three
taken at approximately 2 years of age and then, sessions of limb lengthening, aiming to achieve
4360
Adductor
magnus m.
2.
Pass FL2
through
intermuscular
septum
1.
Pass FL2
under
patellar
ligament
FL1
Fig. 6 The Super Knee procedure. The Super Knee,
utilising the split fascia-lata (FL) to create an intra-
articular anterior cruciate and an extra-articular posterior
cruciate ligament
58 cm at each session of lengthening, and in
addition a contralateral epiphyseodesis may be
required to achieve limb length equality at
maturity.
For the more severe types of CFD hip recon-
struction is not appropriate as there is an absent
femoral head. It type 2B is possible to provide hip
stability by performing a pelvic support
osteotomy but this surgery should be delayed
until approximately 1214 years of age as other-
wise the osteotomy grows out and needs to be
repeated [15]. Having provided pelvic support it
is then possible to undertake lengthening.
In type 3 with a diaphyseal defect prosthetic
reconstruction should be considered, that is
reconstruction with either a Symes amputation
or a Van Ness procedure to aid fitting of an
extension prosthesis. The Van Ness is
a controversial operation. It can provide excellent
function and the ankle joint functions as a knee to
enable wearing of a below knee prosthesis. How-
ever it does give a bizarre appearance to the limb,
C. Bradish
particularly when not wearing the prosthesis,
and it is sometimes said that this procedure is
unacceptable in warmer climates where children
wish to be exposed to the sunshine. With
a Type 3 CFD the distal femur is fused to the
pelvis and a derotation undertaken through the
distal femur (the Paley modification of the Brown
procedure) (Fig. 8).
In this modification a Chiari osteotomy is
performed of the pelvis and this enables
medialisation of the femur and improves the
mechanical axis of the limb. The femur is attached
to the pelvis by two or three screws, two of these
cross the growth plate of the distal femur and
achieve an epiphyseodesis which is important so
that the knee joint, which now functions as the hip,
remains at the level of the opposite hip.
Paley has certainly advanced our understand-
ing of CFD and proposed very logical treatment
options. There does remain concern however
about the magnitude of the surgery and how the
function compares with early prosthetic recon-
struction, particularly with the more severe
Types 1B and 2A. In the UK the National Insti-
tute for Clinical Excellence (NICE) assesses and
gives recommendations with regards to new sur-
gical procedures. In April 2009 they published
recommendations with regards to hip reconstruc-
tive surgery in CFD. Because of the complexity
of the surgery required each case should be
audited and a record kept of clinical outcomes.
They further stated that the procedure should
only be carried out in units that specialise in
limb reconstruction, by surgeons with specialist
knowledge of neonatal hip dysplasias and exper-
tise in limb lengthening procedures.
Fibular Hemimelia
Fibular hemimelia is the commonest long bone
deficiency syndrome. The incidence is between
7 and 20 per million. The aetiology is unknown
but is thought to be due to an embryonic insult at
68 weeks gestation. Although the terminology
refers to the fibula, it is a complex deformity involv-
ing the whole leg to a variable extent. The associ-
ated abnormalities are (from distal to proximal):
(a) Absent lateral rays of the foot
(b) Tarsal coalition producing a valgus hindfoot
Leg-Length Discrepancy in Children 4361

a b
Gluteus
Gluteus
medius m.
maximus m.

Gluteus
Psoas m. medius and
quadriceps 1st
muscle osteotomy
Quadriceps removed
m. from bone
as a unit

c d

3rd osteotomy

2nd osteotomy

e f

Wires with
Proximal femur threaded heads
rotated 135 inserted

Fig. 7 (continued)
4362 C. Bradish

g Gluteus
maximus m. Gluteus
medius m.

Adductor-
quadriceps
tendon unit
sutured to new
greater trochanter

Quadriceps
m.

Fig. 7 Super Hip 2 procedure (With kind permission of the Journal of Bone and Joint Surgery[Am])

Fig. 8 Modified Van Ness rotationplasty in type 3 CFD (With kind permission of the Journal of Bone and Joint Surgery[Am])

(c) An equino-valgus foot position (h) Patella instability

(d) Wedged distal tibial physis producing more
growth on medial side
(e) Partial or complete absence of the fibula
which is represented by a fibular anlage
(a fibrocartilagenous precursor)
(f) Tibial bowing with apex anteromedial and
a dimple at the apex of the bow
(g) Cruciate deficient knee, the anterior cruciate
is frequently absent and less commonly the
posterior
(i) Undergrowth of the lateral femoral condyle
producing a valgus knee
(j) Short femur, this may be any of the Paley
types of CFD
(k) Acetabular dysplasia
(l) Upper limb deformities ranging from syn-
dactyly to deficient ulnar rays to upper limb
amelia.
There are various classifications of this
condition. The most frequently used is that of
Leg-Length Discrepancy in Children
Achterman and Kalamchi [16]. This describes
three types; in types 1A and 1B the fibular
is present, it is totally absent in type 2. In 1A
the proximal fibular epiphysis is distal to the
level of the proximal tibial growth plate and
is often smaller than on the normal side, the
distal fibular growth plate is proximal to
the dome of the talus. In 1B there is partial
absence of the fibula. More recently Stanitski
has introduced a morphological classification
which describes the individual components
of the deformity in each child [17]. In this
classification the fibula is described as
I (nearly normal), II (small or miniature) or
III (absent); the tibiotalar joint and epiphysis
as H (horizontal), V (valgus) and S (spherical);
if there is a tarsal coalition this is represented
by c and finally the number of rays is given.
So for example a child described as IIVc4
would have partial fibular aplasia, a valgus
ankle, a tarsal coalition and a four ray foot.
She confirmed the findings of Achterman that
there is no correlation between the percentage
of tibial shortening and the type of fibula
absence. In addition she found that feet with
three or fewer rays always had a coalition, but
coalitions may also be seen in some four and
five ray feet. The fibula is likely to be absent if
there is a three ray foot. The ankle may be
horizontal with all types of fibula deficiency.
A ball and socket ankle with a spherical dome
to the distal tibia may be seen with Types 1A
and B. In this condition the ankle joint develops
with an articulation permitting both flexion/
extension and inversion/eversion to compen-
sate for a talocacaneal coalition. The ankle is
typically valgus with a deficient lateral tibial
physis where the fibula is Stanitski II or III.
The tibia may have anterior bowing of up to
40 and have a shortening up to 40 %. Achterman
found CFD in approximately 50 % of cases.
The cruciate ligaments of the knee are typically
deficient with longitudinal limb deficiencies. ACL
is typically deficient or absent. An aplastic ACL
with a normal PCL will be found in approximately
40 %, an aplastic ACL and hypoplastic PCL in
approximately 20 % and complete absence of both
cruciates in approximately 20 %. The PCL is never
4363
involved without ACL involvement. Clinical
examimation, and in the older child (>7 years)
knee radiology including tunnel views of the
knee, enables the diagnosis. When both cruciate
ligaments are absent (Fig. 9) the tibial spines
and intercondylar notch will fail to develop. If
just the anterior cruciate is absent there will
be a narrowed notch and hypoplasia of the lateral
tibial spine [18].
In view of the wide range of severity of
fibular hemimelia it is not surprising that there
is an equally wide range of treatment options,
ranging from a shoe raise alone for the mild
deformity to amputation and prosthetic man-
agement for the most severely affected. Herring
and Birch [19] have proposed a functional clas-
sification to aid in the decision making. This is
summarized in the table below
Classification Treatment
Type 1: Functional
foot
05 % inequality Orthosis/epiphyseodesis
610 % Epiphyseodesis and/or
lengthening
1130 % 12 lengthenings (or amputation)
>30 % >2 lengthenings versus
amputation
Type 2: Non
functional foot
Functional upper Early amputation
extremity
Nonfunctional Consider salvage
upper extremity
A functional foot is one which can be made
plantigrade and which has three or more rays. If
an amputation is being proposed it is best under-
taken at approximately 9 months of age so that
the child can learn to walk with a prosthesis.
A Symes or Boyd amputation is recommended
so that an end bearing stump is produced without
the problem of tibial overgrowth which is seen
with a below knee amputation in children.
Other surgeons feel that amputation is virtu-
ally never indicated and better function can be
achieved by preservation of the foot, even if
prosthetic reconstruction is being undertaken.
If undertaking reconstruction the first priority
is the foot. The foot deformity should be
4364
Fig. 9 Absence of cruciate ligaments producing gross
knee deformity. (a) Appearance of the knee with an absent
anterior cruciate ligament and normal posterior. There is
narrowing of the notch and hypoplasia of the lateral tibial
spine. (b) Appearance of the knee with absence of both the
corrected to enable walking with a plantigrade
foot, this may subsequently need protection with
an ankle foot orthosis. Lengthening can be under-
taken at the time of foot correction if this is
delayed until between 18 and 24 months, or post-
poned until there is a 5 cm discrepancy and the
child managed with a raise until then. The foot
should be protected with any lengthening to pre-
vent recurrent deformity. At the time of length-
ening the tibial bowing is corrected. The frame
will need extending to the femur to prevent knee
subluxation if there is significant knee laxity with
absence of both the ACL and PCL. Lengthening
of 58 cm can be undertaken and this will need
to be repeated according to the predicted discrep-
ancy, with further lengthenings at 710 years
and 1214 years. If three lengthening procedures
are undertaken and also an epiphyseodesis
the total limb length discrepancy which can be
corrected is up to 26 cm (one lengthening of 5 cm,
two of 8 cm and a 5 cm correction with
epiphyseodesis). If the femur is also short
additional length can be obtained by undertaking
a femoral lengthening.
C. Bradish
anterior and posterior cruciate ligaments. Note the absent
intercondylar notch and tibial spines. From Manner HM
et al, J Bone Joint Surg 2006,88-A;130-7 With kind
permission of the Journal of Bone and Joint Surgery [Am]
Paley [20] has classified fibular hemimelia
according to the ankle deformity (Fig. 10). Type
I has an essentially normal ankle. In Type II there
is a ball and socket ankle. In Type III there is ankle
valgus which as a consequence of ankle deformity
in IIIa, of subtalar deformity in IIIb and due to
a combination in IIIc. The subtalar deformity is
due to the tarsal coalition creating an abnormal
hindfoot. (He also describes a type IIId with talar
deformity but an absent tarsal coalition).
Type I just requires lengthening, in type II
lengthening is combined with a supramalleolar
osteotomy.
In type IIIa, with an ankle deformity, the foot
deformity will require correction by an opening
wedge supramalleolar osteotomy. Foot correc-
tion should be combined with tibial deformity
correction if there is significant bowing.
There will therefore need to be a second
diaphyseal osteotomy. If such extensive surgery
is being undertaken it should be performed at
1824 months and combined with the first length-
ening. The fibular anlage acts as a deforming
force and will need to be excised (Fig. 11a).
Leg-Length Discrepancy in Children 4365

Paley Classification of FH

Type I - Stable Type II - Dynamic Valgus

LDTA = ADTA =
85 - 90 80 - 85

Type III - Fixed Equino-valgus Type III - Fixed Equino-valgus Type III - Fixed Equino-valgus
a) ankle type b) subtalar type c) combined ankle and subtalar type

ADTA
ADTA 90 ADTA 90 90

Fig. 10 The Paley classification of fibular hemimelia (With kind permission of Dror Paley)

This can be done via a proximal incision over the
peroneal nerve and a distal lateral incision over
the ankle. The peroneal nerve is protected and
then the anlage excised between these two inci-
sions. The tendo Achilles and peroneal tendons
are lengthened. Next the foot is stabilised in its
neutral position with wires passed from the heel
into the distal tibia.
A supramalleolar osteotomy is then per-
formed with correction of the ankle deformity.
The opened wedge is bone grafted with an iliac
crest graft and the wires are passed across
the osteotomy. The tendons are repaired.
The Achilles tendon repair can be augmented
by using the resected fibular anlage. If undertak-
ing lengthening the fixator is then applied.
The illustration shows an Ilizarov fixator
(Fig. 11b), however the Taylor Spatial frame is
ideal for this as it enables accurate simultaneous
lengthening and angular correction and also
correction of any internal rotation deformity of
the foot. The corticotomy is performed at the
apex of the tibial deformity.
In type IIIb the deformity is at the level of the
tarsal coalition and so the corrective osteotomy is
done at this level and then similarly maintained
with wires while lengthening is performed
(Fig. 11c).
In type IIIc both a subtalar and
a supramalleolar osteotomy will be required.
Very occasionally an ankle fusion will be
required.
Functionally the outcome achieved with
a successful reconstruction is virtually identical
to that achieved with an amputation and
a prosthesis [21]. This is both a reflection of the
surgical skills of reconstruction and the advances
in prosthetics. The careers obviously need to be
fully appraised of this to aid their informed deci-
sion making.
The difficulty arises with unsuccessful recon-
struction. Should the child sustain a subluxation
4366 C. Bradish

a Resect
membran
Peroneal N.
1.

2.
Cut

Cut

b 12.
Gradual
lengthening
11.
Apply
fixator

Fig. 11 (continued)
Leg-Length Discrepancy in Children 4367

c Subtalar 3

angulation
translation

5.
Angulation
translation
osteotomy

Fig. 11 (a) Resection of tethering membrane. (b) Exter- of type IIIb (subtalar) (With kind permission of the Jour-
nal fixator and correction of deformity. (c) nal of Bone and Joint Surgery[Am]).
Angulation translational osteotomy. Surgical correction

or dislocation of the knee or should there be Tibial Dysplasia

a persistent ankle valgus or equinus deformity
the reconstructed child will fare less well. The
worst outcome is when an unsuccessful recon-
struction is followed by an amputation at a later
age. The child will then have had the inconve-
nience of multiple surgical procedures and is
likely to achieve an inferior result from an ampu-
tation as he may well have developed knee
stiffness.
It is very important therefore when counsel-
ling the carers that they are fully appraised of the
advantages and disadvantages of reconstruction
versus amputation and understand that
a satisfactory result cannot be guaranteed with
reconstruction. As with reconstruction for CFD,
surgery should only be undertaken by an experi-
enced limb reconstruction surgeon.
Congenital deficiency of the tibia is the rarest of
the long bone deformities, occurring with
a frequency of approximately 1 per million live
births. There are autosomal dominant and reces-
sive forms. It is seen in association with syn-
dromes such as ectrodactyly- tibial dysplasia
(that is the association of cleft foot/hand and
tibial dysplasia) and hand deformities affecting
predominantly the thumb, with bifid or absent
thumbs. There may be duplication of the foot
(diplopodia) and of the hands.
The tibia is absent or dysplastic but the fibula
is intact. In this condition the treatment is largely
determined by the condition of the knee. There
are various classifications. The Jones classifica-
tion [22] recognises four types. In type 1A the
tibia is absent, in type 1B it is initially absent but
4368
subsequently appears on radiographs. In type 2
the distal tibia is not seen, in type 3 the proximal
tibia is not seen (but may subsequently appear on
radiographs), in type 4 there is a tibio-fibular
diastasis. Because of the confusion about the
presence of the tibia the Jones classification was
subsequently modified by Kalamchi and
Dawe [23] and they describe three types, Type 1
is complete absence of the tibia. In Type 2 there is
a relatively normal proximal tibia but there
is distal tibial aplasia. In these children active
quadriceps contraction can usually be elicited.
There is a flexion contracture of the knee. The
proximal tibia may not be seen on initial Xrays
but its presence as an unossified anlage can be
predicted from the normal development of the
distal femur. In Type 3 there is dysplasia of the
distal tibia with a tibiofibular synostosis.
The recommendations until recently were that
a Type 1 should be treated by knee disarticulation
and Type 2 by creation of a tibiofibular synostosis
proximally and a Boyd amputation of the foot.
Type 3 can sometimes be managed prosthetically
otherwise a calcaneofibular fusion and Boyd
amputation is performed.
The Brown procedure whereby the fibular is
transferred to the intercondylar notch of the
femur in order to try and create a knee joint is
unsatisfactory in the long term as it does not
create stability and it makes prosthetic fitting
difficult.
More recently Weber [24] has proposed
a new classification, using MRI to define the
status of the tibial anlage. The tibial anlage is
described as hypoplastic (complete but underde-
veloped), aplastic (partially absent) or totally
absent (agenesis). He describes seven types of
tibial dysplasia; in Type 1 there is hypoplasia of
the tibia, in Type 2 a tibio-fibular diastasis (i.e.,
a Jones Type 4), in Type 3 distal aplasia of the
tibia, in Type 4 proximal aplasia of the tibia, in
Type 5 aplasia of the tibia both proximally and
distally, in Type 6 complete absence of the tibia
with a double fibula and in Type 7 agenesis of
the tibia with a single fibula. His Types 5 and 6
were not categorised by either Jones or
C. Bradish
Kalamchi and these types in Webers series
accounted for approximately 15 % of cases. He
stresses the importance of the cartilaginous
anlage that can be defined by MRI. If the anlage
comes into contact with the corresponding joint
it matures completely to an osseous structure,
including growth plates, because of the process
of callus distraction, weight loading and func-
tional loading. In his series the sex ratio was
equal as was the proportion of right or left
sided involvement. His Type 7 with complete
absence of the tibia accounted for just over 50 %
of cases. In addition he has proposed a new
treatment for this type, utilising the patella to
create a tibial plateau beneath which he achieves
union of the fibula to provide a functional knee
joint and tibia. With the help of two crossed
capsular flaps and a Z-plasty of the quadriceps
tendon, the patella is transposed under the fem-
oral condyles to function as a tibial plateau. The
proximal fibula is fused centrally to the external,
cortical surface of the patella. A mini-ring
fixator construction is used to stabilize the com-
ponents, although permitting mobilization of the
knee arthroplasty in the immediate postopera-
tive period. He reports a satisfactory outcome
[25] in one case although experience of other
limb reconstruction surgeons is awaited.
This technique relies on modern principles of
limb reconstruction and avoids amputation where
there is complete tibial absence.
Post-Operative Care and
Rehabilitation
All children undergoing limb reconstruction will
require meticulous follow up while in any fixator
to detect early and treat any complications. Paley
has divided the post operative problems which
are frequently encountered with limb lengthening
into problems, obstacles and complica-
tions [26]. A problem is a difficulty encountered
during the treatment which can be addressed by
non-operative means and which does not influ-
ence the final outcome. An example would be
Leg-Length Discrepancy in Children
a wire or pin track infection that responds to
antibiotics, or a bone deformity which can be
corrected by adjustment of the fixator. An obsta-
cle requires an unscheduled return to theatre for
its solution, for example premature consolidation
needing a repeat corticotomy or an infected wire
needing replacement. The eventual outcome is
again not affected. A true complication is for
example subluxation of the knee or a fracture
post frame removal which affect the eventual
outcome. Approximately 80 % of patients will
have problems, 20 % obstacles and complica-
tions should occur in less than 10 %. Complica-
tions are more frequently encountered with
congenital deformities rather than acquired, and
are also more commonly seen with both multi
level surgery and with extensive lengthening. It
is for this reason that lengthening is usually lim-
ited to 58 cm, or a maximum of 30 % of the
initial length of the bone. In addition lengthening
of the femur and tibia simultaneously is avoided
to prevent knee stiffness and premature joint
degeneration. Any surgeon undertaking limb
lengthening must be fully aware of the necessity
for a thorough assessment of the child and not just
the short limb, and must also be well versed in
limb reconstruction techniques.
The surgeon is not the only member of the
team, the physiotherapists, occupational thera-
pists and orthotists are also vital to success. Phys-
iotherapy is ideally commenced prior to surgery
so that the child can be taught to walk with
a frame or crutches. It then continues throughout
the time in the fixator to try and maintain mobility
and joint range of motion. The child is mobilised
as soon as possible following surgery, getting up
with crutches on the first post operative day if
possible so that it is realised that relatively nor-
mal life is possible with a frame on the leg and the
frame is not looked upon as the enemy. The
child should be taught to participate in frame
adjustment and cleaning if old enough. Physio-
therapy exercises are undertaken daily while in
hospital and then at least twice weekly following
discharge. It is accepted that a wheelchair will be
required for longer distances, but crutch walking
4369
is encouraged to aid joint function. The child
should walk slowly putting his foot to the ground
to maintain ankle stretch, rather than hopping
along with his crutches with the foot in equinus.
Following frame removal physiotherapy is con-
tinued until normal muscle strength and joint
mobility has been regained. This may take up to
a year. If staged lengthening procedures are
planned the child should have fully recovered
from one before commencing the next.
Orthoses aid the rehabilitation. The shoe can
be raised with 1 cm wedges, secured with Velcro,
which are sequentially removed as the limb
grows. An AFO at neutral to be worn at night
will help to prevent equinus. Springs can be
passed from the shoe lace eyelets to the frame to
similarly help to prevent equinus.
Complications
The description of problems, obstacles and
complications has been mentioned. To avoid com-
plications meticulous attention to detail is required
and a holistic approach to the child, assessing not
just the limb deformity. Despite this all limb recon-
struction surgeons will encounter some complica-
tions and it should therefore be fully explained to
the carers (and child if old enough to understand)
that, particulary if extensive lengthening is being
proposed, that joint stiffness, bowing or fracture of
the regenerate and an outcome deemed less than
satisfactory sometimes occur.
Summary
The diagnosis and treatment of lower limb defor-
mities is described. This is challenging but very
rewarding surgery. Our understanding of the
aetiology of congenital deformities is advancing
and hopefully in the future there will be new treat-
ment techniques which are less surgically invasive
and rely on biological techniques for limb recon-
struction. Until that time arrives the best results are
obtained by a dedicated limb reconstruction team.
4370
References
1. Lyons K, Ezaki M. Molecular regulation of limb
growth. J Bone Joint Surg (Am) 2009;91:4752.
2. Paley D. Principles of deformity correction. 3rd ed.
Berlin: Springer; 2005. p. 118.
3. Shapiro F. Developmental patterns in lower extremity
discrepancies. J Bone Joint Surg (Am) 1982;64:63951.
4. Paley D. Principles of deformity correction. 3rd ed.
Berlin: Springer; 2005. p. 695716.
5. Kenwright J, Albinana J. Problems encountered in leg
shortening. J Bone Joint (Br) 1991;73B:6715.
6. Winquist RA, Hansen ST, Pearson RE. Closed
intramedullary shortening of the femur. Clin Orthop
Rel Res. 1978;136:5461.
7. Catagni MA, Lovisetti L, Guerreschi F, Combi A,
Ottaviani G. Cosmetic bilateral leg lengthening: expe-
rience of 54 cases. J Bone Joint Surg Br. 2005;
87-B:14025.
8. Bradish CF. Cosmetic bilateral lengthening. Further
opinion. J Bone Joint Surg. 2006;87-B e-publication.
9. Aston WJS, Calder PR, Baker D, Hartley J, Hill RA.
Lengthening of the congenital short femur using the
Ilizarov technique: a single-surgeon series. J Bone
Joint Surg. 2009;91-B:9627.
10. Oh CW, Song HR, Kim JW, Choi JW, Min WK, Park
BC. Limb lengthening with a submuscular locking
plate. J Bone Joint Surg. 2009;91-B:13949.
11. Rozbruch SR, Fragomen AT, Ilizarov S. Correction of
tibial deformity with use of the Ilizarov-Taylor spatial
frame. J Bone Joint (Am) 2006;88:15674.
12. Davies R, Holt N, Nayagam S. The care of pin sites
with external fixation. J Bone Joint Surg (Br) 2005;87-
B:7169.
13. Rogala EJ, Wynne-Davies R, Littlejohn A, Gormley J.
Congenital limb anomalies: frequency and
aetiological factors: data from the Edinburgh Register
C. Bradish
of the Newborn (196468). J Med Genet
1974;11:22133.
14. e-presentation by Paley D on Congenital Short Femur at
http://limblengtheningdoc.org/ortho_conditions.html
15. Rozbruch SR, Paley D, Bhave A, Herzenberg J.
Ilizarov Hip Reconstruction for the late sequelae of
infantile hip infection. J Bone Joint Surg (Am)
2005;87:100718.
16. Achterman C, Kalamchi I. Congenital deficiency of
the fibula. J Bone Joint Surg. 1979;61-B:1337.
17. Stanitski D, Stanitski C. Fibular hemimelia, a new
classification system. J Pediatr Orthop. 2003;23:304.
18. Manner HM, Radler C, Ganger R, Grill F. Dysplasia of
the cruciate ligaments; radiographic assessment and
classification. J Bone Joint Surg. 2006;88-A:1307.
19. Herring JA, Birch JG. The child with a limb defi-
ciency. Rosemont: American Academy of Orthopae-
dic Surgeons; 1998.
20. e-presentation by Paley D on Fibular Hemimelia at
http://limblengtheningdoc.org/ortho_conditions.html
21. Walker JL, Knapp D, Minter C, Boakes J et al. Adult
outcomes following amputation or lengthening for
fibular deficiency. J Bone Joint Surg. 2009;91A:
797804.
22. Jones D, Barnes J, Lloyd-Roberts GC. Congenital
aplasia and dysplasia of the tibia with intact fibula.
Classification and management. J Bone Joint Surg (Br)
1978;60-B:319.
23. Kalamchi A, Dawe R. Congenital deficiency of the
tibia. J Bone Joint Surg. 1985;67-B:5814.
24. Weber M. New classification and score for tibial
hemimelia. J Child Orthop. 2008;2:16975.
25. Weber M. A new knee arthroplasty versus brown pro-
cedure in congenital total absence of the tibia: a prelim-
inary report. J Pediatr Orthop (Br). 2002;11:539.
26. Paley D. Problems, obstacles and complications of
limb lengthening by the Ilizarov technique. Clin
Orthop. 1990;250:81104.