Congenital Pseudoarthrosis and Lengthening

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MINI-SYMPOSIUM:ILIZAROVTECHNIQUE

(i) Congenital pseudoarthrosis and lengthening

A.Khaleeland R.D.Pool

Department of Orthopaedics, St Peters Hospital, Guildford Road,Chertsey, Surrey KT 16 OPZ, UK

Abstract Limb lengthening and the treatment of congenital pseudoarthrosisrequire the ability to predictably regenerate bone in vivo. Ilizarov, through his experi-ments inthe1950s and1960s, summarised thisin hisPrinciple of TensionFStress: livingtissue, when subjected to slow steady traction, becomes metabolically active in bothbiosynthetic and proliferative pathways, a phenomenon dependent on vascularity andfunctionaluse.

Limb lengthening and the treatment of congenital pseudoarthrosis as well as

other selected orthopaedic conditions, are based on this principal.This article outlinesthe management of congenital pseudoarthrosis and limb lengthening problems usingIlizarovs techniques.c 2003 Elsevier Ltd. All rights reserved.

CONGENITALPSEUDOARTHROSIS

This is a rare disease of unknown aetiology and unpre-

dictable course. It can present in one of several forms

and may appear at birth or, more often, within the first

2 years of life.

Based on the plain X-rays of 25 cases of congenitalpseudoarthrosis of the tibia, Anderson in1973 described

four presentations with distinct clinical and prognostic

features. Boyd1 in 1982, working independently, pre-

sented his classification, which had six subtypes based

on histological and radiological characteristics (Table 1

and Fig.1).

Patients with X-rays showing bone endresorbtion fol-

lowed by sclerosis, who then resorb bone graft rapidly

post-operatively, have a poor prognosis. Those with

cystic lesions have a better prognosis.

The relationship between pseudoarthrosis and neuro-

fibromatosis was first described by Ducroquet and Cot-

tard in 1937. Since then authors have reported a variableassociation in 38 --70% of cases. Contrary to Andersons

postulation, there does not appear to be a direct rela-

tionship between neurofibromatosis and the ultimate

functional result of congenital pseudoarthrosis.

Theproblems

Both Hatzoecher (1708) and Paget (1891) have been cred-

ited with having described the first case of congenital

pseudoarthrosis. To date, it remains one of the most

difficult orthopaedic conditions to treat.

Essentially there are three problems and the most

fundamental of these is bony. There is dysplasia of bone

distally leading to segmental weakness, anterolateral

bowing and subsequently a pathological fracture. The

fracture site contains no callus but rather hamartoma-tous tissue, and the fracture does not unite.

The next major problem is that of deformity. This

consists of leg length discrepancy, multilevel, multidirec-

tional tibial deformity, proximal migration of the fibula,

ankle mortise valgus, ankle dorsiflexion and cavo-valgus

foot. These deformities can result in significant disability

even if bony union is secured.

The natural history of the disease is variable

and the next problem is that of recurrence in the

form of refracture. Whilst the risk of refracture di-

minishes with skeletal maturity, it is never completely

eliminated.

Management

The treatment of established congenital pseudoarthrosis

is surgical. The primary aim of treatment is to achieve

bony union at the pseudoarthrosis site.

Coleman has suggested the outlines of a successful

treatment program: to achieve and maintain bone and

joint alignment, achieve andmaintain effective osteogen-

esis; and to promote and enhance longitudinal bone

growth.

ARTICLE IN PRESS

Correspondence to: AK, 3 Endsleigh Gardens,Walton onThames KT12

5HE,UK. Fax: +44-1932- 429- 005; E-mail: [email protected]

Current Orthopaedics (2003) 17, 411-- 417

c 2003 Elsevier Ltd. All rights reserved.

doi:10.1016/j.cuor.2003.10.003


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The surgical options available to treat establishedpseu-

doarthrosis include internal fixation, usually with bone

graft, external fixation with the Ilizarov2 frame, the tech-

nique of free vascularised fibular graft or amputation.

Internal fixation

This usually involves complete excision of the pseudoar-

throsis, application of bone graft and fixation with an

intramedullary rod. When the distal fragment is small,

the rod may be introduced from the heel, as described

by Charnley. Umber3 recommended using dual tibiofibu-

lar intramedullary fixation and advocated immobilisation

in a hip spica for 6 months. However, Coleman suggest

that after dual rod application patients should be encour-

aged to walk sooner.The results of intramedullary rodding and bone graft-

ing have ranged from 54% to 67% success rates in achiev-

ing bony union. Refracture occurs with growth of the leg

as the end of the rod migrates proximally. Stiffness in the

ankle and subtalar joint may occur and this can result in a

longer lever arm across the pseudoarthrosis, increasing

the risk of refracture.Transfixing the distal tibial growth

plate may further compromise bone growth.

Paterson and Simonis4 successfully treated 24 out of

30 pseudoarthroses with excision of the pseudoarthro-

sis, complete correction of tibia and fibula deformities,

intramedullary fixation and autologous bone graft. Theyadded electrical stimulation through an implanted bone

growth stimulator delivering a direct current of 20 mA.

However Pho et al.5 did not consider electromagnetic

stimulation useful in treating severe pseudoarthrosis or

those with deformities. Currently, electromagnetic in-

duction remains at best an adjunct to primary bone

treatment.

Free vascularised bone graft

Taylor, Miller and Ham first described this microvascular

technique of filling a tibial defect in1975. It is a demanding

procedure that involves the transfer of vascularisedbone, usually the fibula, as bone graft following complete

excision of the pseudoarthrosis. The harvested graft is

either fixed with screws or intramedullary wires.

The procedure relies on hypertrophy of the fibula,

which at this age is predictable.Other advantages of this

technique include the ability to correct deformity and

ARTICLE IN PRESS

Figure1 Boydtype III congenitalpseudoarthrosis.

Table1 Anderson and Boyd classification of congenitalpseudoarthrosis

Anderson Boyd

Club foot Club foot a ssociated with

anterior tibial angulation

I Anterior b owinga ssociated

with congenital deformitiesCystic Cystic changes in the tibia II Anterior bowing and hourglass

tibial constriction associatedwith neurofibromatosis

Dysplastic Anterolateraltibialbowingwith

dysplastic segmentalchanges

III Cystic changes in thetibia

Sclerotic Anterior a ngulation withsclerotic changes of bone

IV Sclerosisofthetibiawithoutnarrowing

V Dysplasia of the f ibula with orwithouttibialpseudoarthrosis

VI IntraosseousneurofibromaorSchwannoma, with or withoutpseudoarthrosis

412 CURRENTORTHOPAEDICS


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any leg length discrepancy. There is however significant

donor site morbidity including tibial fracture and valgus

instability of the ankle. The most common problems of

free vascularised grafting are non-union at one end of

the fibular transfer and progressive or residual tibial de-

formity. In Wielands series of19 patients treatedwith mi-

crovascular fibular transfer, 18 had healed at an averagefollow-up of 6 years. There were five non-unions after

the index operation and these required nine secondary

procedures. Furthermore, 10 tibiae had deformity in the

form of valgus and or AP mal-alignment despite bracing

to skeletal maturity. Two out of nine patients had a late

refracture.

Some authors recommend a vascularised bone graft

only for large tibial defects (43 cm) or shortening of

45 cm. Simonis et al.6 suggests that patients with small

defect should be treated with a conventional bone graft-

ing, intramedullary nailing and electrical stimulation and

not with vascularised fibular bone graft.

Ilizarov technique

Clinically, based on mobility at the pseudoarthrosis

site, Ilizarov7 classified pseudoarthrosis as stiff or

mobile. Ilizarov considered that mobility reflects the

nature of the tissue between the bone ends. When the

pseudoarthrosis is stiff, the tissue between bone ends is

dense fibrous or fibrocartilagenous tissue whereas in

mobile pseudoarthrosis it is either loose connective

tissue or a true synovial pseudoarthrosis. The relevance

of this classification is that a stiff pseudoarthrosis can be

made to form bone by distraction while a mobile pseu-doarthrosis cannot. Mobile pseudoarthrosis can in some

cases be converted to stiff pseudoarthrosis by applying

compression, which results in tissue transformation,

and subsequently distraction can be applied to the result-

ing stiff pseudoarthrosis to achieve bony union.

Using the Ilizarov tensioned wire fixator, four basic

principles can be applied to the management of pseu-

doarthrosis: single level compression; compression fol-

lowed by distraction; simultaneous compression and

distraction and lateral transposition of bone into bony

defects.

According to Ilizarov, closed treatment (i.e. withoutopen resection) should be reserved for primary, pre-

viously untreated pseudoarthrosis; whereas previously

operated cases, should be treated with open radical re-

section of the pseudoarthrosis with either acute short-

ening and apposition of bleeding bone ends and leg

length correction or gradual bone transport to fill the

defect.

In order to reduce the risk of refracture, the cross-

sectional area of bone at the site of pseudoarthrosis is

maximised. Closed treatment should only be chosen

when this area is already wide; however, there are a few

techniques of widening this area of non-union. By simply

overlapping the two ends of the non-union and applying

side-to-side compression doubles the overall diameter of

bone at the site of pseudoarthrosis. Alternatively one

end of bone may be inserted into the other either by

closed or even open method to increase the cross-sec-

tional diameter of the healing bone. Angular deformities

are corrected acutely if there is no contracture and thepseudoarthrosisis mobile or when open resectionis per-

formed.

In pseudoarthrosis treated by closed methods, defor-

mity should be corrected gradually using appropriate

hinges to preventneurovascular injury.Whilst correcting

deformity gradually, it is important to have two levels of

fixation within each bone fragment. Distally, fixation to

the foot may be necessary when the distal tibial frag-

ment is small. Leglength can be correctedby distraction,

either through a corticotomy, through an open physis or

even through the pseudoarthrosis. Ilizarov was the first

to use physeal distraction and still recommends thistechnique in young children because this not only gener-

ates the widest cross-sectional area in the bone but also

growth plate closure is rare. Lengthening through the

pseudoarthrosis is usually reserved for those with a stiff

pseudoarthrosis and pre-existing wide bone ends. Alter-

natively, sub-physeal corticotomy can be performed

2--3 mm distal to the proximal tibial physis.

Cortical distraction is carried out at a rate of 1mm

a day but physeal distraction is limited to 0.5 mm a day.

Similarly, distraction through the pseudoarthrosis is car-

ried out initially at 0.5 mm a day until new bone is seen

and then increased to1mm a day (Figs. 2 and 3).

The integrity of the fibula can be re-established usingthe frame. Transporting the lateral malleolus distally

using an olive wire achieves this, and the resulting fibula

defect is closed by proximal corticotomy and fibula

transport. Gracheva et al.8 performed fibular transport

in 40 out of 89 cases. The advantages of restoring the

fibula integrity are not only to reduce the loads across

the tibia and provide improved resistance to torsional

stress but also to normalise the ankle by reducing

ARTICLE IN PRESS

Figure 2 Stiffpseudoarthrosistreatedwithclosed distraction.

CONGENITAL PSEUDOARTHROSIS AND LENGTHENING 413


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the slope of the distal tibial physis andpreventing a valgus

ankle.

In a multicentre study Paley et al.

9

reported theresults of treatment of 16 pseudoarthroses of the tibia

treated using Ilizarov principles. Closed treatment was

achieved in six patients, open reduction performed in

another six and resection of the pseudoarthrosis in

three, of whom one had bone transport and the other

two had acute shortening and lengthening through a

proximal corticotomy. Fifteen out of the 16 cases went

to unite after initial treatment. The mean time of treat-

ment was 5.6 months. Four patients had a mal-union.

Two out of the 16 patients had a late refracture. Overall

10 secondary procedures were required in six patients.

These authors were able to identify two causes for

refracture.They found that persistent angular deformity,particularly at the pseudoarthrosis site, contributes to

refracture and suggest that all angular deformities be

corrected. In particular those deformities in the distrac-

tion zone should be corrected before consolidation of

the regenerate. The other cause of refracture was pin

site stress fracture. Keeping wires as far away from ab-

normal bone as possible can prevent this, they suggest.

The obvious advantage of the Ilizarov method in this

condition is its ability to treat pseudoarthrosis compre-

hensively, addressing simultaneously the problems of ti-

bial non-union, bone defects, deformity, limb length

discrepancy, fibular non-union and proximal migration,ankle valgus and foot deformities.The apparatus consist

of 1.5 mm K wires, which are able to provide stable fixa-

tion even in very small bone fragments and in the

presence of osteoportic bone without the need for

intra-articular fixation.

Refractures, when they do occur can be treated by

exactly the same technique. All other methods of treat-

ment are still possible if the Ilizarov method is aban-

doned. The disadvantage of the Ilizarov frame is related

to the use of the external fixator. Generally, external

fixators are less well tolerated than internal devices and

pin track infections are a common problem.

Amputation

The role of amputation in congenital pseudoarthrosis

has been controversial, with some authors feeling that

there is no indication for amputation.However, in every

patients course the surgeon must evaluate the potential

benefits of amputation. This is essentially the predict-able, early, excellent functional result with a below knee

amputation vs the cost of limb salvage both to the child

and family in maintaining a useless deformed limb.

McCarthy10 outlined the indications and level of

amputation for pseudoarthrosis. While maintaining

that each case is considered individually, a failure

to achieve bony union after three surgical attempts,

significant leg length discrepancy (45 cm), interference

with growth distal to the pseudoarthrosis, and an

unreasonable amount of time under medical care would

constitute indications for amputation. He suggests

that the level of amputation be distal to the pseudoar-

throsis, using the technique for a cartilage preservingSymes amputation. Amputations at the level of the pseu-

doarthrosis result in poor stump configuration, bony

overgrowth and poor skin coverage while amputations

proximal to the pseudoarthrosis provide a very short

stump and a decreased lever arm.

LIMBLENGTHENING

Leg length inequality is a common problem, with some

23% of the general population having a discrepancy of

1cm or more. Fortunately major congenital limb defi-ciencies are rare. Limb length discrepancy in the upper

limb is usually not a functional problem. The distraction

technique described for leg lengthening can also be ap-

plied in congenital upper limb deficiencies for lengthen-

ing and correction of angular deformity.

Sophisticated methods of imaging allow more accu-

rate assessment of the deficiency whilst advances in re-

construction, particularly using the circular fixator, have

made reconstruction rather than amputation possible

particularly in the milder forms of deficiency. However,

the surgeon must remember that in severe cases recon-

struction cannot produce a normal limb.This is perhaps

the hardest thing to explain to patients and parents. Inmajor deficiencies, frequently amputation and a modern

prosthesis is still the best advice.

History

Codivilla of Bologna in1905 published the first account of

lengthening of the lower limb shortened due to injury,

disease or malformation. His method of distraction and

transfixion to gain limb length continued for many years,

albeitwith modifications.Credit for the first skeletal dis-

traction, however, goes to Lambert of Lillie but in 1939

Abbott and Saunders reported on their lengthening

ARTICLE IN PRESS

Figure 3 Mobile pseudoarthrosistreatedwithopenresectionand either bonetransport or acute shortening andlengthening.



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procedure using external fixation and incremental

distraction.The Wagner technique of open, rapid, bone

lengthening and bone grafting has fallen out of favour

because of the need for multiple procedures and high

risk of complications.

Currently, the DeBastiani technique and the Ilizarov

method of bone lengthening using either a dynamicmonolateral fixator or a multiplanar circular frame with

tensioned wires respectively, combined with gradual dis-

traction are the methods of choice for leg lengthening.

Aetiology

Leg length inequality is most commonly due to shorten-

ing of one leg but occasionally it may be due to lengthen-

ing. The numerous causes of leg lengthening can

be broadly divided into two categories, congenital or

acquired.11--13

Congenital conditions include those with associatedfocal deficiencies such as proximal focal femoral defi-

ciency, longitudinal deficiency of the fibula (the most

common cause) and longitudinal deficiency of the tibia.

Congenitalconditions with unilateral lengtheninginclude

idiopathic hemihypertrophy, neurofibromatosis, Klippel--

Trenaunay--Weber syndrome and Proteus syndrome.

Some skeletal dysplasias are associated with leg length

discrepancy; these include multiple enchondromatosis,

multiple osteochondromatosis, and fibrous dysplasia.

Acquired causes of unilateral reduction in leg lengthin

children include trauma, physeal injury secondary to in-

fection, paralysis, disuse, ischaemia, radiation and burns.

Acquired causes of unilateral increased leg length in chil-dren include accelerated physeal growth secondary to

fracture, arteriovenous fistulae other vascular lesions,

joint inflammation (juvenile rheumatoid arthritis or hae-

mophilia), and symphathectomy.

Studies of gait analysis confirm that asymmetry of gait

requires leg length inequality of at least 2 cm or 3%.The

association between leg length inequality and back pain

has not been established.14 There does not appear to be

an increase in back pain in patients with small leg length

discrepancy but Tjenstrom and Rehnberg reported im-

provement in back pain in patients who had large leg

length discrepancies corrected (3--14 cm). They also re-ported few lower extremity complaints in patients with

leg length inequality.There is no evidence that leg length

inequality leads to early hip or knee arthrosis.11

Assessment

Clinical assessment should include determination of a le-

vel pelvis with the patient standing. Leg length inequality

is thususually apparent; however, its location maynot be

obvious. It is important that the patient stands withboth

knees and hips extended and with no abduction/adduc-

tion or rotation. Blocks of various heights placed under

the short leg to level the pelvis indicate the amount of

inequality. This is a more accurate method than using

tape measure.

Various radiographic methods have been used to mea-

sure leg length discrepancy including teleroentgenogram

(single exposure AP radiograph with a ruler); orthora-

diography (three separate exposures to reduce magnifi-cation errors) and scanography which uses a similar

technique but exposure size is reduced and all three ex-

posures are on a single film. Presently CT scanogram is

the method of choice.However, all these techniques be-

come inaccurate in the presence of hip andknee contrac-

tures and separate radiographs of each limb segment

may have to be done.

In skeletally immature patients with leg length discre-

pancy, accurate prediction of growth is essential for pre-

operative planning: surgery performed too late and the

discrepancy may not be sufficiently corrected, or too

early, with over correction. The factors to consider forappropriate surgical timing include the skeletal age; the

projected time to skeletal maturity; the amount of

growth inhibition of the shortened limb; and the antici-

pated leg length discrepancy at skeletal maturity.

Once the current leg length inequality has been mea-

sured, a prediction of the ultimate inequality at skeletal

maturity is needed to determine the treatment regime.

The methods used include the simple arithmetic method,

which assumes the growth of the distal femur at 1cm

per year and proximal tibia growth at 0.6 cm annually

and a skeletal maturity age of 16 for boys and 14 for girls.

The growth remaining graphs relate chronological and

skeletal ages to limb length in order to predict theamount of future growth. Moseleys straight-line graph

which takes into consideration the childs growth centile

and the degree of growth inhibition in the shorter leg, is

used to predict the effects of corrective surgery and to

plan the timing of surgery. Often, these methods are

used in combination.

Management

Treatment objectives need to be carefully considered

and the decision to treat depends not only on the

amount of leg length discrepancy but also on the func-tional requirements. Usually the goal is to obtain leg

length equality or within 1cm, produce a level pelvis and

improve function. In some situations some leg length in-

equality (Table 2) is beneficial, for example, children with

neuromuscular disorders may use the inequality to aid in

floor clearance during the swing phase of gait.

Surgical treatment

Surgery for limb length inequality can be directed to the

pelvis, femur or tibia. Generally the choice depends on

the site of inequality, surgeons preference and underlying

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conditions.Typically the shortened segment (tibia or fe-

mur) is lengthened or the opposite corresponding seg-

ment shortened. Occasionally these rules are not

followed, a tibial lengthening may be performed in a pa-

tientwith hip dysplasia despite the femur being the short

segment; this is done to prevent hip subluxation during

lengthening.

Epiphysiodesis

This procedure is obviously carried out in the skeletally

immature. Distal femoral and proximal tibial epiphysiod-

eses have been the most commonly used procedures for

equalisation of predicted leg length discrepancies of be-

tween 2 and 5 cm at maturity. The procedure reliably in-

hibits bone growth but requires accurate prediction of

final leg length inequality and the degree of growth inhi-

bition to achieve the desired result. As it is carried out

on the normal longer leg it may be difficult to convince

the patient and parents that the operation is indicated.

It is also useful, in conjunction with a limb lengthening

procedure, to reduce the total length discrepancy. Anadditional indication for percutaneous epiphysiodeses is

partial growth plate ablation to correct angular defor-

mity. Currently the most common method is percuta-

neous drilling under image intensification and it results

in physeal closure in 85 --100% of patients with few com-

plications. Epiphyseal stapling produces a temporary ar-

rest of longitudinal growth by staples, when the leg

length discrepancy is corrected, the staples are re-

moved, and growth can resume. Staples must be placed

perpendicular to the growth plate; the periosteum and

epiphyseal vessels should be left intact to avoid perma-

nent growth.

Shortening

This accurate technique can be used after skeletalmatur-

ity. Recovery from shortening procedures is usually

shorter than lengthening procedures.Generally, femoral

shortening is preferred to tibial shortening, as larger re-

sections are possible. Femoral shortening can be per-

formed by open or closed methods at the

subtrochanteric, mid-shaft, and supracondylar levels,

and the osteotomy fixed by plating or intramedullary

nailing. After femoral shortening, quadricep weakness

with extension lag of the knee and genu recurvatum due

to hamstring weakness is common.

In skeletally mature patients, tibial shortening is not

widely practised. Broughton et al. reported good results

using a step-cut method; some patients developed neu-

rovascular disorders or loss of function. Tibial fixation

can be a problem and locked intramedullary nailing has

been used, but complications include infection, calf mus-

cle weakness, axial deviation, and problems with union.

Lengthening

The method of choice for limb lengthening is callus

distraction. Through his experiments Ilizarov demon-

strated the ability to predictably form bone by

distraction osteogenesisFbone formation by intra-

membranous ossification under ideal conditions.

This technique can result in lengthening of 25% or

more but typical length gained is about15% or 6 cm.The

limits of lengthening are related to patient tolerance,

bony consolidation, functional range of movement andjoint stability above and below distraction.

Generally about 30 days are required in the frame for

distraction and consolidation per centimetre of length

gained. Though Ilizarov found the metaphyseal region

the ideal site for corticotomy, the level of the corticot-

omy does not appear to have a significant effect when

used in the tibia. The mid-diaphysis appears to be the

ideal corticotomy site in the femur. Large leg length

inequalities, can be treated by staged lengthenings or

simultaneous ipislateral femoral and tibial lengthening

combined with appropriately timed epiphyseodesis.

Combined femoral and tibial lengthening requires ag-gressive soft tissue management to prevent contractures

and joint subluxation.

In the traditional Ilizarov technique, low energy corti-

cotomy is followed by a period of latency (about 7--10

days) that allows the healing process to begin. Distrac-

tion is then carried out in fractionated doses (1mm per

day) through the use of a stable, circular external fixator

using self-tensioning K wires. Once the appropriate

length is gained, the frame is maintained through a peri-

od of consolidation, which allows the regenerate to re-

model. Throughout the treatment it is vital that the

limb undergoes physiological loading.

ARTICLE IN PRESS

Table 2 Treatmentoptions forlimblength inequality

Leglength inequality Treatment

o2 cm nothing or shoe raise2--5 cm epiphysiodeses or shortening procedure6--15 cm lengthening procedure415 cm multiple staged lengthening, amputation



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Whilst the basic biological principal remains un-

changed, several techniques have evolved in terms of

frame choice.

The De Bastiani technique utilises a monolateral

external fixator for distraction. This is easier to apply

and better tolerated by the patient but does not allow

for correction of deformity. Because of the cantilevernature of the construct, it can produce angular defor-

mity in the regenerate. Overall, monolateral fixators

have the same results as circular frames for modest

lengthening.

In order to reduce the period of external fixation

and reduce deformity, limb lengthening can be carried

out over an intra-medullary nail. This is usually done in

skeletally mature patients and involves over-reaming

of the medullary canal and performance of a cortico-

tomy before inserting a straight rod, that is initially

locked proximally. An external fixator is then applied

with wires or pins either anterior or posterior to therod. After the distraction period, the external fixator is

removed and the rod is locked distally. The two major

problems with this technique are the high risk of infec-

tion (up to15%) and medialisation of the mechanical axis

of the bone as the bone is distracted along its anatomic

axis (over the rod).This can be a problem with large dis-

tractions.

A number of designs for totally implantable intra-

medullary devices are being developed. Such devices

would eliminate the need for external fixation. The

patient has to be skeletally mature with no bony

deformity and the medullary canal sufficiently wide.

Distraction usually requires between 31 and 91 ofrotation of the limb and this can be accurately measured

magnetically.

Limb-length surgery is exceptionally challenging. Nu-

merous complications occur, frequently, and the rates

vary depending on the degree of lengthening, definition

of complication, surgical experience and on underlying

pathology. Major complications of fracture, infection

and joint subluxation can reach 25% even in experienced

hands. Some high-risk circumstances have been identi-

fied, such as patients with a congenital aetiology, (who

have more than twice the major complication rate of

those with an acquired aetiology); where the percentagelengthened is greater than 15%; simultaneous lengthen-

ing of femur and tibia of congenital aetiology and length-

ening of congenitally short femurs.15

CONCLUSION

Based on years of experimental and clinical work,

Ilizarov offered a method of solving the challenging

orthopaedic problem of bone deficiency. External

fixation currentlyis themethod of choice for limblength-

ening. It would be wise however, to remember his wordswhat seems, at first, a simple procedure can lead to cat-

astrophic outcome if applied with insufficientknowledge

of the basic principles and techniques of application.

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Congenital Pseudoarthrosis and Lengthening