Current Orthopaedics (2008) 22, 139e149

ava i lab le at www.sc ienced i rec t . com

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CHILDREN

Clubfoot

Stephen James Cooke*, Birender Balain, Cronan Christopher Kerin,Nigel Terrence Kiely

Robert Jones and Agnes Hunt Orthopaedic Hospital, Oswestry, Shropshire SY10 7AG, UK

KEYWORDSClubfoot;Congenital talipesequinovarus;Aetiology;Assessment;Management

* Corresponding author.E-mail addresses: s.j.cooke@virgin

yahoo.com (B. Balain), cronankerin@nigel.kiely@rjah.nhs.uk (N.T. Kiely).

0268-0890/$ - see front matter ª 200doi:10.1016/j.cuor.2008.04.002

Summary

Clubfoot or congenital talipes equinovarus is a common congenital abnormality of uncertainaetiology. It presents with fixed cavus, adductus, varus and equinus of the foot. Serial manip-ulation and casting using the Ponseti technique can produce a plantigrade, pain-free, func-tional foot in the majority of cases in the long term. Most patients treated this way willrequire only minimal surgery, such as Achilles tenotomy, dramatically reducing the need forextensive, open releases. Recurrent and complex clubfoot can also be treated by the Ponsetitechnique but some challenging cases still require surgical correction. Those that resist pri-mary or revision operations can be salvaged by arthrodesis but the long term results are lesspredictable. This review will summarise the current theories on aetiology and pathogenesis,assessment and management according to the Ponseti regime, surgical options for primaryclubfoot and recurrences and possible future directions.ª 2008 Elsevier Ltd. All rights reserved.

Introduction

Clubfoot or congenital talipes equinovarus is one of the mostcommon congenital orthopaedic abnormalities. It can beisolated or associated with other serious congenital abnor-malities, especially if bilateral and severe. The ideal aim oftreatment is to achieve a functional, pain-free, plantigradefoot in the long term. Over recent years, and since this topicwas last reviewed in this journal,1 there has been a generaltrend away from extensive open releases towards serial ma-nipulative techniques coupled with minimal surgery, such as

.net (S.J. Cooke), balainb@doctors.org.uk (C.C. Kerin),

8 Elsevier Ltd. All rights reserved

the Ponseti technique. A small number of resistant casesmay benefit from primary surgery and complex recurrencesstill present considerable management difficulty.

Aetiology

As with many medical conditions, clubfoot has a multifac-torial aetiology and probably represents an end-point ofseveral disease processes.

Genetics

Clubfoot occurs in approximately 1 per 1000 live births inthe UK (0.1%), with Polynesians being affected six timesmore commonly. The male to female ratio is 3 to 1 and up to50% are bilateral. There is a monozygotic twin concordance

.

140 S.J. Cooke et al.

of 33%2 and if one sibling has clubfoot the chance of a secondbeing similarly affected rises nearly 30 times to 3%.3 Theo-ries vary between involvement of a single major gene, pos-sibly on the long arm of chromosome 2,4 and polygenicinheritance patterns. These observations imply a geneticcomponent, but other factor(s) may be more important.

Embryology

There are three main phases of foot development in utero.In the initial phase (between weeks 5 and 6) the foot beginsto develop in line with the leg. The embryonic stage (from 6to 7 weeks) is characterised by a phase of fibular growth.The lateral part elongates relative to the medial and thefoot adopts a ‘clubfoot-like’ posture. During the final foetalphase (8 to 9 weeks), the tibial side of the leg and footdevelops, correcting the position to that observed in thenormal newborn.5 It has been suggested that an interrup-tion during the foetal phase of growth results in a clubfootdeformity. The time of onset and duration of that interrup-tion affects the overall severity. Several agents have beenimplicated such as a chemical teratogen, viral infection, ra-diation and hormonal imbalance but no definite cause hasyet been identified. Observations of the seasonal variationin the incidence of clubfoot have leant some support to anenvironmental or viral aetiology, but other studies havefound no correlation.6

Soft tissues

There have been many studies looking at the macroscopic,microscopic and ultrastructural appearance of the softtissues involved in the clubfoot. The muscles, tendons,ligaments, nerves and blood vessels all demonstrate abnor-malities compared to normal tissue. These changes areundoubtedly linked and it is not yet fully understood which,if any, are driving forces in the development of clubfootand which are secondary.

Some anatomical studies have shown anomalous muscles(eg accessory soleus) and/or tendons, but these findings areinconsistent and certainly do not occur in all cases. A morecommon finding is an absent or small anterior tibial arteryand dorsalis pedis. This abnormality is also found inassociation with fibular hemimelia and may representgrowth arrest during the late embryonic phase of growthmentioned above. It is also possible that this vascularinsufficiency is the primary cause of clubfoot7 and studiesof foetal anatomy have noted that these vascular abnor-malities are more prominent in early foetal life aroundthe time of foot development.8

The leg muscles, especially the posterior and medialgroups, in a child with clubfoot tend to be smaller in girthand shorter in length when compared with normal and thetendon of tibialis posterior in particular is often severelythickened.9 The degree of shortening is proportional to theseverity of clubfoot. The muscles have a higher proportionof connective tissue and the ratio of type I to type II fibers isincreased, both of which are signs of neurogenic atrophy.10

Ponseti has linked the degree of in vitro protein synthesiswith the severity of clubfoot. Muscle cells with very highlevels of collagen synthesis and low levels of non-collagen

protein synthesis occur in children with severe clubfoot.This gradually returns to normal by age 6 to 7, the age afterwhich clubfoot is very unlikely to recur.

The ligaments and joint capsules on the posterior andmedial side of the foot and ankle are thickened and thoselaterally tend to be thin and weak. There is an increase ofcollagen fibers in the thickened tissues with high numbersof mast cells, fibroblasts and myofibroblasts,11 appearancessimilar to scar tissue. This has led to the retraction fibrosistheory of clubfoot aetiology12 analogous to cicatrisation ofscar tissue. It also explains the continuing deformity seen inrecurrences.

Anatomy

The four main anatomical abnormalities can easily beremembered by the mnemonic ‘CAVE’ e Cavus, Adduction,Varus and Equinus (Fig. 1). Cavus is an increased height ofthe vault of the foot and in clubfoot is due to pronationof the forefoot in relation to the hindfoot, with plantarflexion of the first ray. Although the whole foot appearsto be in a supinated position, this forefoot/hindfoot rela-tionship is integral to understanding the initial correctivestep required by the Ponseti technique (see below). The cu-neiforms and metatarsals are adducted but of normalshape. The midfoot is adducted, primarily at the talo-navic-ular joint. The talus and navicular are wedge shaped andthe navicular is medially displaced (in severe cases it nearlyabuts the medial malleolus) and inverted such that it is inan almost vertical position. The calcaneus is severely plan-tarflexed, medially displaced and inverted below the talussuch that it lies below and almost in line with the talus.This accounts for the equinus and varus deformities andfor the reduced AP and lateral talo-calcaneal angles seenon x-ray (Fig. 2). The ankle, although in a plantarflexedposition, is relatively normal.

As mentioned above, the posterior and medial structuresare short and thick. The calcaneo-navicular (spring),deltoid and talo-navicular ligaments along with the tibialisposterior tendon hold the foot in an adducted position. Theposterior talo-fibular, posterior calcaneo-fibular, posteriorand medial talo-calcaneal and posterior tibio-talar liga-ments along with tightness of the gastro-soleus complexcontribute to the equinus and varus. Since the insertion ofthe tendons has medialised, there is medial displacementof tibialis anterior and the long toe extensors. These cannow act as deforming forces, pulling the midfoot andforefoot into further adduction and inversion.

The motion of the calcaneus under the talus has beendescribed as a mitered hinge, a hinge at 45� to thehorizontal rotating around a fixed axis. This is somewhatsimplistic in that the subtalar joint has no fixed axis butrather is a kinematic chain whose movements depend uponthe inclination and curvature of the involved joint surfacescoupled with dynamic capsular, ligamentous and musculo-tendinous forces. The entire tarsus moves as a unit andmovement in anatomical directions (adduction, abduction,flexion, extension, inversion and eversion) cannot beseparated. Thus movement of one part of the foot in-variably causes movement elsewhere, which may be ina different plane or direction. Varus movement of the

Figure 1 A clubfoot model (MD Orthopaedics, Iowa, USA) showing the deformities from the front (A), lateral (B) and medial (C)sides. TH e talar head. NO e calcaneocuboid joint.

Clubfoot 141

calcaneus, for example, actually comprises medial rota-tion, flexion and inversion of the calcaneus. The cuboid andnavicular follow into adduction and inversion in front of thetalus. Reversal of this movement by reducing the navicularinto its normal position, forces the calcaneus to derotate,abduct and evert below the talus. This understanding formsthe basis for the Ponseti method of manipulation andcasting described below.

Assessment

The assessment of an infant with clubfoot falls into 4 parts,antenatal diagnosis, examination, assessment of severityand investigations.

Antenatal diagnosis

Though commonly diagnosed post-natally, prenatal ultra-sound diagnosis is possible and can help with parentalcounselling and organising tertiary referral such thattreatment can commence as soon as possible after birth.The positive predictive value of antenatal ultrasound isover 80% with almost no false negatives and even theseverity of clubfoot can be estimated, although lessaccurately13 Studies have identified that in many patients

the foot deformity is not isolated. As many as 2/3 may beassociated with at least one other abnormality, althoughthis is often minor and may be unrelated to the club-foot.14,15 In bilateral and/or severe cases of clubfoot, thereis a higher incidence and severity of associated anomalies.Urogenital abnormalities, neural tube defects, cardiac de-fects, arthrogryposis and other musculoskeletal anomaliesaccount for the majority. Abnormal karyotypes are presentin 5e10% (XXY, XXX, trisomies 18 and 21) and although notroutinely used in this country, some specialists recommendamniocentesis if clubfoot is diagnosed. Clubfoot can beseen as early as 12 weeks of gestation and up to 2/3 havean associated polyhydramnios16 This contradicts the histor-ical belief that clubfoot is due to intrauterine mouldingwhich occurs well after 20 weeks. There is no associationbetween clubfoot and hip dysplasia; in fact, there may bea negative association.

Examination

The examination of the child with clubfoot should be headto toe, systematically looking for associated anomalies. Inparticular, the spine should be inspected along witha neurological examination, and all the joints assessed forstiffness characteristic of arthrogryposis.

Figure 2 Plain AP and lateral radiographs of the immaturefoot. The talo-calcaneal and talar-1st MT angles are shown. Onthe AP view (A), the normal talo-calcaneal and talar-1st MT an-gles are approximately 25� and 0� respectively. On the lateralview (B), the normal talo-calcaneal is approximately 45�.

Table 1a Pirani score, (hindfoot)

‘LOOK’ 0 No heel creasePosterior

crease0.5 Mild heel crease1 Deep heel crease

‘FEEL’ 0 Hard heel (calcaneum in normal position)Empty

heel sign0.5 Mild softness1 Very soft heel (calcaneum not palpable)

‘MOVE’ 0 Normal dorsiflexionRigidity of

equinus0.5 Foot reaches plantigrade with knee

extended1 Fixed equinus

142 S.J. Cooke et al.

The important factor is to distinguish flexible, positionaltalipes from true clubfoot. In the former, the deformity isfully correctible with no fixed equinus. Severe metatarsusadductus can be confused with clubfoot, but again, there isno fixed equinus and no hindfoot deformity. These condi-tions are associated with moulding and care should betaken to examine the infant’s hips.

Assessment of severity

There is a definite relationship between the severity ofclubfoot and the number and severity of associated

abnormalities. Severe or complex clubfeet are more likelyto require extensive surgery, are more prone to recurrencefollowing treatment and have a somewhat poorer outcome.For these reasons it is important to have a method ofassessing and documenting clubfoot severity. It is alsodesirable to monitor the effect of interventions. Severalscoring systems are in use, for example, the Dimeglio score,the Carroll severity scale and the Pirani score. All of thesesystems have been independently validated. Inter- andintra-observer reliability is very good and they correlatewell with patient-based assessments of outcome.17

The Pirani score is commonly used, and for simplicity wehave only included a detailed description of this sys-tem.18,19 It consists of 6 parts, each of which can havea score of 0, 0.5 or 1, giving a total score from 0e6. Themore severe the clubfoot, the higher the score. Examina-tion can be divided into ‘look, feel, and move’ and is sepa-rated into hindfoot and midfoot components. Each consistsof 3 of the 6 components to give a hindfoot score and a fore-foot score of 0e3 (Tables 1a and 1b).

Investigations

Though many radiological modalities have been used, inroutine practice no formal investigations are required. Thebones of the newborn are mostly cartilaginous with onlysmall ovoid ossification centers present in the calcaneumand talus. This makes assessment of the axes and thusangular relationships difficult to determine. The position ofthe infant’s foot and x-ray plate is crucial and can bedifficult to replicate accurately. This is illustrated by a poorcorrelation between angles measured by plain radiographyand those measured by 3D CT.20 The correlation betweenradiological and clinical outcomes is variable and certainlysurgery is not indicated to correct radiological abnormality.Decisions on initial treatment are therefore made purely onclinical grounds. Ultrasound and MRI have been used to as-sess clubfeet and monitor the response to treatment. MRI inparticular has elegantly shown the gradual correction of de-formity seen when treating clubfoot conservatively.21,22

It is important to remember that even in well treatedclubfoot some radiological abnormalities persist longterm.23 The tarsal bones tend to be smaller in volume andcharacteristic flattening of the talar dome is common. Re-search-based investigations, such as pedobarography andelectromyography reinforce this point.

Table 1b Pirani score, (midfoot)

‘LOOK’ 0 No deviation from straight lineLateral border

of foot0.5 Medial deviation distally

1 Severe deviation proximally‘FEEL’ 0 Reduced talo-navicular jointTalar head 0.5 Subluxed but reducible

talo-navicular joint1 Irreducible talo-navicular joint

‘MOVE’ 0 No medial creaseMedial creasea 0.5 Mild medial crease

1 Deep creasealtering contour of foot

a The foot should be moved to the position of maximum cor-rection when assessing the medial crease.

Clubfoot 143

Management

The goals of treatment are to achieve a plantigrade, pain-free, functional foot for the life of the patient. The footshould be cosmetically acceptable, have good mobility andrequire no specialised footwear or orthoses once treatmentis complete. Parents should be informed that in the vastmajority of cases this is achievable but the foot will neverbe entirely normal.

The two main methods of management in use today arethe Ponseti technique and the French physical therapytechnique. When done well, both have excellent long termresults and avoid major surgery in the majority of patients.In this country the Ponseti regime is being increasingly usedand this is described in detail below.

The Ponseti technique

At 40 years follow-up, 78% of patients treated by thismethod can be expected to have a good or excellentfunctioning foot in the long term. This compares with 85%of subjects without clubfoot, assessed using the sameoutcome measures. 93% will require an additional pro-cedure, usually Achilles tenotomy alone or combined withtibialis anterior transfer.24

Treatment should begin as early as possible and consistsof manipulation and serial plaster casts changed at weeklyintervals. At each cast change, the foot is examined and thePirani score noted. The manipulations follow a specificprotocol which aims to correct all deformities simulta-neously except equinus (Fig. 3).

The forefoot must be brought into the correct relation-ship with the midfoot. There is pronation of the forefootwith plantarflexion of the first ray. The forefoot is thereforesupinated by lifting the first ray (which makes the overalldeformity appear worse initially) and gently abducted usingcounter pressure against the lateral border of the talarhead. No pressure must be placed around the heel or in theregion of the calcaneo-cuboid joint as this will blockcalcaneal movement (Fig. 4A). With subsequent plastersthe forefoot, now in its correct relationship with the mid-foot, is gradually brought from supination to neutral whilst

increasing the amount of abdution. The talo-navicular,calcaneo-cuboid and subtalar joints are simultaneously re-duced. The calcaneum will evert, abduct and rotate underthe talus to assume its normal position without any directmanipulation of the hindfoot whatsoever. By the last plas-ter, the foot should be in approximately 70� of abduction.Each plaster is initially placed below the knee and themoulding performed. It must then be completed to thegroin with the knee in 90� flexion. The ligaments are grad-ually stretched in plaster and the immature, mostly carti-laginous, tarsal bones begin to remodel.

Once the foot is in a position of abduction and the heel isin valgus, the equinus can then be addressed. It isimportant to dorsiflex the entire foot as pressure underthe forefoot can cause dorsiflexion at the midtarsal jointsand a rocker-bottom deformity (Fig. 4B). The majority ofpatients will require percutaneous Achilles tenotomy. Themore severe the initial Pirani score the more likely theneed for tenotomy18 If the foot cannot be brought to atleast neutral with the knee flexed then a tenotomy is per-formed. This can be done under local anaesthesia but inour center a general anaesthetic is preferred. A blade ispassed anterior to the tendon, turned posteriorly and thetendon divided from deep to superficial. The foot shouldbe able to reach 15� of dorsiflexion. A further cast is appliedand left for 3 weeks.

The total number of plasters varies from 4 to 10, moreseverely affected feet generally requiring longer treatment.The severity as assessed by the Pirani score should graduallyreduce at each examination (Fig. 5). Usually the forefootscore reduces first, followed by the hindfoot score. If the de-formity is not improving or if more than 10 casts are requiredthen conservative measures can be said to have failed.

Following successful serial casting the infant is placed into a ‘boots and bar’ splint. This consists of a soft sandalholding both feet in 70� of abduction and 15� of dorsiflexionconnected by a bar of approximately shoulder width. Thismust be worn at all times, except bath time, for 10 weeksfollowed by night and nap time use until 4 years of age.

The French technique

This method, described by Bensahel,25,26 involves regulargentle manipulations of the foot in a relaxed child and ad-dresses the deformities in a very similar manner to Ponseti.Individual treatments last approximately 30 minutes andare done daily for 2 weeks then twice weekly until correc-tion is achieved. This takes approximately 6 to 8 weeks forthe cavus, varus and adductus and up to several months tocorrect the residual equinus. A flexible splint is worn in be-tween sessions. In his hands, Bensahel reports a 93% good orexcellent outcome at skeletal maturity with only a 23% op-eration rate, although surgery tended to be more extensivethan simple Achilles tenotomy. More severe cases of club-foot were associated with longer treatment times, a higherrate of surgical intervention and a poorer outcome.

The complex clubfoot

A minority of feet, approximately 5%, do not respond to thePonseti or French techniques described above. The

Figure 4 Pressure on the calcaneocuboid joint rather than the talar head leads to a midfoot break (A). Although the foot mayappear corrected, the talo-navicular joint is not reduced and the hindfoot is not corrected. Forced dorsiflexion of the forefoot canlead to a rocker-bottom deformity (B).

Figure 3 Sequence of pictures showing the Ponseti method of clubfoot correction. The 1st ray is elevated correcting the align-ment of the forefoot (A). In this position, the heel remains in varus (D). The forefoot is then brought in to progressively greaterdegrees of abduction with counter pressure against the talar head (B, C). The calcaneus corrects to neutral (E) and finally valgus(F) without any direct manipulation of the heel itself.

144 S.J. Cooke et al.

Figure 5 Clinical photographs of an 8 day old boy with isolated unilateral clubfoot (A, B). The same patient after 3 (C) and 5 (D)weeks of Ponseti treatment.

Clubfoot 145

deformities are very severe at presentation, scoring 5.5 or 6on the Pirani scale. All of the metatarsals are in plantaris,signified by a deep transverse plantar crease in the sole ofthe foot, and the great toe appears to be short andhyperextended. The equinus is severe and fixed, witha short, thickened, medially displaced Achilles tendon.Primary surgery in this group is technically difficult andoften has poor results. Ponseti has published a modificationto his standard regimen which reports good results after anaverage of 2 years follow up27 Instead of just dorsiflexingthe first ray, the whole foot is grasped and by pushing upon all the rays with both thumbs, the plantaris can be seri-ally corrected. Nearly all patients in this series required atleast one Achilles tenotomy with 6% needing a second.Complications occurred in 22% but were mostly minor andrelated to cast problems. Due to the short follow-up, it isnot yet known whether these feet will do well in the longterm or go on to require more extensive surgery.

Recurrent clubfoot

Roughly 1/3 of patients will suffer a relapse and the mainpredictor of recurrence is non-compliance with the splint.28

Interestingly, the severity of deformity at presentationdoes not seem to be a factor. 80% of recurrences occur inthe first 2 years of life27 with the majority of the rest occur-ring up to age 6. Approximately 5% occur after this time,a third of which may have a previously undiagnosed neuro-muscular condition such as Charcot-Marie-Tooth disease ormyotonic dystrophy.29 Stiff, recurrent clubfeet may be theonly sign of a distal arthrogryposis. The child with recurrenttalipes should therefore be thoroughly re-examined. Pon-seti casting should be restarted straight away and contin-ued until correction is re-achieved. Further Achillestenotomy or tibialis anterior transfer should be consideredas indicated. Parents must be educated regarding the

146 S.J. Cooke et al.

importance of splintage and the vast majority of recurrentclubfeet can be successfully corrected.

Tibialis anterior transfer, whereby the tendon is movedfrom its insertion into the medial cuneiform and firstmetatarsal to the lateral cuneiform, is indicated for re-current clubfoot as the tibialis anterior has a supinating andvarising action. The foot must initially be corrected byrepeat Ponseti plastering and the child must be overapproximately 2.5 years of age, when the lateral cuneiformis ossified. A straight incision is made from the medialcuneiform 5 cm proximally along the line of the tendon.The tendon is divided distally and mobilised back to the ex-tensor retinaculum, which is left intact. A second small in-cision is made overlying the lateral cuneiform anddissection carried out medial to extensor digitorum longus,through extensor digitorum brevis down to bone. The ten-don is passed subcutaneously and inserted through a holedrilled in the lateral cuneiform. It can be secured overa button in a manner similar to flexor tendon repair tothe terminal phalanx of the finger. The leg is placed in anabove knee plaster in the corrected position for 4 weeksand then the suture and button are removed. There is noneed for a splint if this procedure is successful, making ita good option in the cases when boots and bar cannot betolerated.

Late presentation

Late presenting or neglected clubfoot is fortunately un-common in the UK. There are studies showing that clubfoottreatment can start up to 6 months following birth with nochange in the overall outcome30 and a group in Brazil havesuccessfully treated neglected clubfeet in children as old as9 years using the Ponseti method.31 In this study, the lengthof time required in plaster was longer, averaging 4 months.A third of patients required a posterior release to allow fullcorrection of the hindfoot deformity. A 3 year follow-up ofthese patients showed excellent early results but long termfunction is not yet known.

Primary surgery

For this condition, primary surgery refers to proceduresmore extensive than Achilles tenotomy or tibialis anteriortransfer. Due to the success of manipulative and plasteringregimens the number of surgeons performing major primaryclubfoot surgery has reduced dramatically.32 That said, sur-gery is indicated where conservative measures have failedor in syndromic feet. There are very many procedures de-scribed for clubfoot correction involving either the soft tis-sues, the bones or both, making objective evaluation oftheir relative long term efficacy extremely difficult. Resultsare varied and although no randomised controlled trialexists, the outcomes may be poorer in those undergoing ex-tensive surgery compared with conservative manage-ment.23 Short term results can be very good. However50% have demonstrable osteoarthritis of the subtalar jointby 30 years with a similar percentage having a poor overallresult. Stiffness is very common and some patients alsocomplain of muscle weakness, pain and residual defor-mity.33 Others have found that long term results are much

better34 and the more severely deformed feet have an im-proved prognosis if operated on before 3 months, whenthere is a greater potential for remodelling. This contra-dicts another study suggesting the outcome is improved ifsurgery is delayed until 12 months as early surgery is asso-ciated with severe scarring and high chance of recur-rence.35 Heterogeneity between studies makes directcomparison difficult.

Once the decision has been made to operate, the type ofsurgery required must be ascertained. Bensahel has pop-ularised the ‘a la carte’ approach to clubfoot surgery,36

similar to the ‘progressive surgical approach’ coined byGeroge Simons37 i.e. release or lengthen only those struc-tures which are tight and contributing to the deformity.There are three main approaches in use, the posterome-dial, the Cincinnati hemi-circumferential, and the two-inci-sion approach. The three main releases are posteromedial,plantar and lateral.

Once the soft tissues have been addressed, the jointsshould be able to be held in their normal or near normalrelationships. Intraoperative x-rays will help determine ifthis is the case. The bones are not of a normal shape andtherefore acute correction in this manner will be associatedwith joint incongruity. Temporary fixation with k-wires issometimes necessary to hold the bones whilst remodellingoccurs and the foot should be placed in plaster in thecorrected position, although this may endanger the bloodsupply. The time in plaster will depend on the age of thepatient and the extent of surgery performed but will usuallybe for 4 to 6 weeks. If present, the wires are then removedand splintage commenced.

Posteromedial soft-tissue release

This release is used for residual equinus and hindfoot varus.The tendo-achillis is divided (age< 1year) or lengthened(age� 1 year). If the equinus has been present for a longtime then capsulotomy of the ankle and subtalar jointsalong with release of the posterior inferior tibio-fibular,the posterior talofibular and the calcaneo-fibular ligamentsmay be required. The superficial deltoid ligament is dividedalong with the talo-navicular joint capsule. The tibialis pos-terior tendon is lengthened and the flexor tendons releasedfrom their sheaths, or lengthened if very short and largeenough to repair.

Plantar release

For cavus deformity, a plantar release is done. The abductorhallucis is released from its origin on the calcaneus; pro-vided dissection is carried out superior to this muscle, themedial plantar, lateral plantar and calcaneal neurovascularbundles will be protected. The plantar fascia, plantarcalcaneo-navicular ligament and short toe flexors are di-vided at their origins and the calcaneo-cuboid and talo-navicular joint capsules are released medially and inferiorly.

Lateral release

It is seemingly counterintuitive to consider lateral releasegiven the anatomy of clubfoot deformity. The lateral

Clubfoot 147

structures are usually attenuated and thin compared to thethickened, contracted medial tissues. However a lateralapproach, in addition to the releases described above,allows capsulotomy of the lateral parts of the talo-navicularand calcaneo-cuboid joints. This provides further mobilityof the subtalar and midfoot joints where posteromedial andplantar releases have been inadequate.

Complications

Serious complications are uncommon and depend verymuch on the type of surgery performed. Avascular necrosis(AVN), infection, overcorrection, pain, stiffness and re-currence have been reported. It must be noted howeverthat all of these, with the exception of AVN, have occurredwhen using the Ponseti method.

Overcorrection may be the result of overzealous primarysurgery and is more likely to happen in children who haveevidence of generalised ligamentous laxity.38 The hindfoot isin valgus with a flattened medial arch. Management of thiscondition should follow the normal sequence with orthoticsbeing first line and surgical options considered later as symp-toms dictate. The added complication of a scarred and pos-sibly multiply-operated foot must be taken into accountwhen counselling patients prior to major corrective surgery.

Revision surgery

Despite best efforts, some clubfeet resist primary treat-ment, whether it is conservative or surgical, and these casespresent considerable management difficulty. The numbersare small and long term comparative studies lacking. Thechild may have an associated neuromuscular condition andtherefore the soft tissues do not respond to surgery in thenormal manner. Another cause may be inadequate releaseat primary surgery. If the child is still less than 2 years old,repeat soft tissue release can be done as described above.Over the age of 2, it is likely that bony surgery will beneeded in addition. Major osteotomies should be reserveduntil the foot is more mature, after 4 years of age. Allchildren and parents must be warned that the long termoutcome is variable with many patients requiring furthercorrective surgery or arthrodesis in the future.

The osteotomies described in the literature are numer-ous but can be broadly classified in terms of which de-formity they aim to correct.

Cavus

Residual cavus can be treated by midtarsal osteotomy. Thiscan be an inferior opening wedge, superior closing wedge ordome type (Akron osteotomy),39 which also allows forefootadduction to be corrected. It is usual to perform the osteot-omy through the midfoot bones but more distal osteotomiesat the level of the tarso-metatarsal joints have beendescribed.

Adductus

Adduction occurs primarily at the talo-navicular and calca-neo-cuboid joints, but also at the tarso-metatarsal joints.

Combinations involving shortening of the lateral column(Dillwyn-Evans procedure) and/or lengthening of the me-dial column address the former and metatarsal osteotomiescan be performed to address the latter. The lateral columncan be shortened by excising a wedge of bone and fusingthe calcaneo-cuboid joint. As the child grows, the medialcolumn will lengthen relative to the lateral and thus furthercorrection achieved.

An opening wedge osteotomy of the medial cuneiform,thus lengthening the medial column, has the advantage ofnot shortening a foot which is already small as part of theclubfoot deformity. Only limited correction can be attainedby this method alone.

Varus

Residual hindfoot varus can be corrected by calcanealosteotomy. The Dwyer osteotomy40 is an oblique, lateral,closing wedge osteotomy that will correct varus and someequinus. More recently, a curvilinear calcaneal osteotomyhas been described which brings the centre of rotation(about which the correction is done) closer to the true cen-tre of deformity. This has achieved good results in terms ofdeformity correction as measured radiologically but it is tooearly to say whether this will be reflected in long term clin-ical results.41

External fixation

All of the deformities can be potentially corrected by theuse of external fixators. Both the Ilizarov system and theTaylor Spatial Frame have been used for clubfoot correc-tion. It is usually combined with either a midfoot orcalcaneal osteotomy, although purely soft-tissue Ilizarovcorrection has been performed in children up to 8 years oldwith good early results.42 During correction it is vital to ob-tain lateral radiographs of the ankle to ensure that the dis-tal tibial physis is not being distracted. Early complicationsare frequent, often relating to pin site problems. Late com-plications include spontaneous ankylosis, recurrence of de-formity and requirement for surgical arthrodesis. A recentpaper with almost 5 year follow up of recurrent clubfeettreated using Ilizarov frames showed a 45% rate of sponta-neous ankylosis, 31% had recurrence of deformity and 37%of feet went on to require arthrodesis. Approximately 75%had an acceptable result clinically after an average of 56months.43 Another study with 6½ year follow up reported85% of patients had fair or poor results with over 50% requir-ing revision surgery.44

Salvage surgery

Salvage surgery for clubfoot relies upon arthrodesis ortalectomy. Talonavicular, triple and pan-talar arthrodesiscan be performed depending on the individual patient, thesymptoms and the pathology. Wedges of bone can beremoved at the same time thus correcting residual de-formity and fusing the joints simultaneously. Talectomy cancorrect hindfoot but not midfoot deformity and allowssome movement at the new joint between the tibia andcalcaneum. It may be of particular benefit in children with

148 S.J. Cooke et al.

primary or recurrent clubfoot as a result of arthrogryposisor spinal dysraphism. A 20 year follow up of patients whounderwent talectomy for clubfoot has shown that 75% havea plantigrade foot that is pain-free whilst walking. How-ever, 2/3 of these patients required additional surgery, 1/3had radiological evidence of tibio-calcaneal arthritis and29% had spontaneous fusion of the tibia to the calcaneum.45

Future directions

There is much ongoing research into the actual causes ofclubfoot, current theories of which have been discussed. Sofar, management is directed at treating the establisheddeformity, but in the future it may be possible to influenceclubfoot development and thus prevent its occurrence.

There have been trials using Botox as an adjunct toconservative management. Early results are promising andwhen combined with the Ponseti regimen may reduce theneed for Achilles tenotomy.46 Further refinements to thePonseti technique include a new custom-fit dynamic ortho-sis that has been shown to increase compliance, the mostimportant factor in preventing recurrence.47

The Bulgarian technique is a newly described procedurethat helps to correct the hindfoot deformity at the time ofpercutaneous Achilles tenotomy. For very stiff, fixedequinus the Achilles tendon is divided in the anaesthetisedinfant. A ‘cats-paw’ retractor is then inserted through theskin of the posterior heel into the calcaneum. Severalminutes of traction pulls the calcaneum down into thecorrect position better than Achilles tenotomy alone. Earlyresults are encouraging.48

Conclusion

1. Clubfoot occurs in 1 per 1000 live births in the UK.2. 50% are bilateral and 5% are ‘complex’. These cases are

more often associated with other congenital abnormal-ities, especially neural tube defects, arthrogryposis,abnormal karyotypes and urogenital abnormalities.

3. It is multifactorial in aetiology with a definite geneticcomponent combined with intrauterine factors, but isnot a result of fetal moulding.

4. Pathological tissues on the medial side are short andthick with high proportions of fibrous tissue and myofi-broblasts e the ‘retraction fibrosis’ theory.

5. The deformities are cavus, adductus, varus and equi-nus. The forefoot is pronated in relation to the hind-foot but the foot is in supination as a whole.

6. Management consists of full history and examinationfollowed by instigation of a conservative corrective re-gime starting as soon as practicable.

7. The aims are to achieve a pain free, functional, planti-grade foot in the long term.

8. The Ponseti technique corrects the cavus, adductusand varus simultaneously with serial plaster casts fol-lowed by ‘boots and bar’ splints 23 hours per day for10 weeks then night time and naps for 4 years. Equinuscorrection requires achilles tenotomy in the majority ofpatients. Approximately 80% will have a good long termresult.

9. Recurrence occurs in up to 1/3 patients and should betreated by re-examination for associated conditions,repetition of the conservative regimen plus repeatAchilles tenotomy or tibialis anterior transfer (afterthe age of 2.5) as indicated. Non-compliance withsplints is the major risk for recurrence and their neces-sity should be reinforced. Recurrence should not beconsidered a failure of conservative management.

10. Complex clubfoot can still be treated conservativelyusing a modification to the Ponseti regimen.

11. Primary surgery should only be considered if conserva-tive techniques fail to correct the clubfoot. It shouldbe as minimal as possible and address those tendons,ligaments and joint capsules which are tight and con-tributing to the deformity e the ‘a la carte’approach.

12. Revision surgery entails repeated or further soft tissuereleases usually combined with one or more osteoto-mies (after the age of 4) to correct residual deformity.Gradual correction with external frames can be done.Results are variable with many going on to spontaneousankylosis or requiring further salvage surgery in thefuture.

13. Salvage procedures are fusion or talectomy. Roughly 3/4 can expect to have a foot that is pain-free at rest andfor walking in the long term but most require more than1 major operation to achieve this.

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