Clubfoot

Clubfoot is a congenital deformity of the lower extremity occurring at 1 to 2 per 1000 births,

making it one of the most common congenital birth defects. 1,2 Clubfoot may occur in
isolation, be syndromic, or be associated with other congenital malformations or
deformations. Untreated clubfoot may cause severe deformity of the lower limb, with related

pain and gait disturbance, often resulting in diminished quality of life and ability to work. 3,4
Early recognition and treatment are crucial to management. The Ponseti method is the most
common treatment method, consisting of manipulation and casting, Achilles tenotomy in

∼90% of cases, followed by bracing for several years. 5,6 Significant surgical interventions are

still required in 3% to 5% of cases for residual or recurrent disease. 7 Despite good long-term
outcomes of modern treatment, achieving them requires substantial medical and parental
resources. Consequently, measures that could minimize the risk of developing clubfoot would
be of great utility.

Classification
Clubfeet were divided into four general classes based on their cause and response to
treatment.8

Postural – benign, resolves completely with stretching and casts.

Idiopathic – true congenital clubfoot of variable severity.
Neurogenic – as seen in spina bifida.
Syndromic – associated with other anomalies. These feet are often quite rigid.

Etiologi of idiopathic clubfoot

Syndromic clubfoot may be seen in patients with distal arthrogryposis, myotonic dystrophy,
myelomeningocele, trisomy 18, amniotic band syndrome, or other disorders. In 80% of cases,
there is no known associated condition; however, a subclinical disease process has long been

suspected. 2,5 There have been many theories as to the cause of idiopathic clubfoot in patients.
Some have considered it to be an arrest in otherwise normal fetal development; others
speculated that there were intrinsic abnormalities in the tendon or bone. There are data to
support a neuromuscular dysfunction with associated vascular and skeletal changes, although
the root cause is uncertain.9 Because 25% of patients report a positive family history, there is

certainly a genetic component. Dobbs and Gurnett 2 have published studies that have led to
improved hypotheses. One possible model for clubfoot is a polygenic inheritance model with
a dimorphic sex threshold. Genes that have been implicated in the pathway include those in
the PITX1–TBX4 transcription pathway.2

Pathogenesis of idiopathic clubfoot

The following have been implicated in the pathogenesis of clubfoot: early developmental
arrest of the foot, primary germ plasm defect in the talus, primary dislocation of the talo–
navicular joint, muscle imbalance due to a developmental neurological deficit, medial
retraction fibrosis, mechanical factors such as intrauterine moulding and oligohydramnios,
and vascular insufficiency.8 In a recent paper, Ippolito et al. 10 suggest a mechanism of muscle
growth impairment as a possible pathogenic factor.

Table 1. Most common and clinically relevant risk factors for clubfoot11
Risk factors
Family history
Selective serotonin uptake inhibitors
Amniocentesis
Maternal/paternal smoking
Maternal obesity
Gestational diabetes

Treatment
The aim of treatment is to produce and maintain a plantigrade, supple foot that will function
well. Historically, there are several methods of treatment but conservative management with
serial casting is now considered the most suitable. Relapse is common and continued bracing
is required for the first 4 years of life to minimize this risk. Babies with associated
neuromuscular disorders and other congenital conditions have a higher rate of recurrent
deformities.12

Timing of clubfoot treatment

It is generally believed that clubfoot correction should begin as soon as possible. 13 Iltar et al
prospectively studied the impact that the age of the patient at the onset of treatment had on
outcome. They found that infants whose cast treatment began after the first post-natal month
achieved better clinical outcomes than those whose cast treatment began before the first
month. They speculated that the less satisfactory results in newborns may have been related
to difficulty with casting or bracing a very small foot.13

CONSERVATIVE TREATMENT
Treatment should begin early, preferably within the first 2 weeks after birth. This consists of
manipulation repeated weekly, with the foot held in place with a plaster cast. The procedure
is the brainchild and result of a lifetime of dedicated work to finesse the program by Dr
Ignacio Ponseti, whose name is synonymous with the procedure.12

The three main components of the deformity are corrected in the following order. Firstly, the
first metatarsal is elevated to correct the cavus and create a flat longitudinal medial border of
the foot. Next, the forefoot is abducted in the plane of the metatarsals which rotates and
everts the hindfoot and corrects the forefoot out of supination. This manoeuvre will also
result in dorsiflexion of the os calcis as it rotates from underneath the talus. Finally, equinus
is corrected by dorsiflexing the foot at the ankle joint. It is necessary in approximately 85%
of cases to perform a percutaneous Achilles tenotomy in order to fully correct the equinus. 12

In Ponseti’s technique, the first cast consists of supination of the forefoot. In this way, the
cavus is treated and the forefoot/midfoot unit becomes reduced. The bony congruity between
the forefoot and midfoot allows for the unit to be moved en bloc. Subsequent maneuvers
consist of abduction or external rotation pressure on the forefoot with counterpressure on the
lateral head of the talus. The forefoot/midfoot unit therefore can move the calcaneus from
varus to valgus, through indirect pressure, with the talus as the fixed point. The metatarsus
adductus and heel varus are corrected simultaneously. Once the heel is in valgus (some care
must be taken in very young infants not to overcorrect, although Dr Ponseti did not believe
overcorrection was possible) tibiotalar dorsiflexion can be addressed through sequential casts
or sectioning of the Achilles tendon.5
Atypical feet have been described by Ponseti and co-workers and must be recognized from
the more usual idiopathic feet because a modification of the casting technique is required.
Clinically, complex club feet were defined as having rigid equinus, severe plantar flexion of
all metatarsals, a deep crease above the heel, a transverse crease in the sole of the foot, and a
short and hyperextended first toe. The Achilles tendon was exceptionally tight and fibrotic up
to the middle of the calf. During casting the forefoot will not correct into 70 degrees of
abduction but advances to approximately 30 degrees only. In this position the os calcis is
corrected under the talus, and this can be felt by careful digital palpation with the pulp of the
index finger alongside the talar head with the finger-tip pressing on the corrected anterior os
calcis. There is midfoot cavus across the width of the foot and not just the first ray so
dorsiflexion is achieved by elevation of the medial and lateral rays. An Achilles tenotomy is
always required in these feet and occasionally may need to be performed earlier than usual
with continued casting after it to ensure complete correction.12

OPERATIVE TREATMENT
Surgery is rarely required for the early correction of the club-foot deformity. The rate of
surgical intervention has fallen to below 5% from the 80% needed in the pre-Ponseti era. The
objectives of club-foot surgery are: (1) the complete release of joint ‘tethers’ (capsular and
ligamentous contractures and fibrotic bands); and (2) lengthening of tendons so that the foot
can be positioned normally without undue tension. A detailed knowledge of the pathological
anat- omy is a sine qua non.12

The tibialis posterior, flexor hallucis and longus tendons as well as the Achilles tendon are all
Z lengthened. The talonavicular and subtalar joints are released, as is the posterior ankle
joint. The peroneal sheath is released and the calcaneofibular ligament divided. The anterior
third of the medial deltoid ligament and the talocalcaneal ligaments are retained to prevent
overcorrection. The procedure can be performed through a posteromedial approach, through a
curved posterior approach known as the ‘Cincinatti’, or through two incisions plantarmedial
and posterolateral. The foot is put in a cast for 8–12 weeks and K-wires can be used to aid
maintenance of correction.12

LATE OR RELAPSED CLUB FOOT

Late presenters often have severe deformities with secondary bony changes, which are
fortunately not at all common in the developed world but sadly more common in the
developing world. The Ponseti programme has been run successfully across many developing
areas of the world with very good success, and the rate of late presentation is reducing as a
result. The first sign of relapse is usually a loss of dorsiflexion and, if allowed to continue, the
foot will adopt the equinus and varus posture that it began with. The other common
occurrence is the development of dynamic supination of the forefoot due to relative
overactivity of the tibialis anterior muscle in the presence of weak peroneals. During the
swing phase of gait the forefoot can be seen to supinate and a tendon transfer is necesary if
this progresses. Most contemporary children’s foot surgeons will transfer the whole tendon to
the lateral cuneiform once the ossific nucleus has developed, usually between the ages of 3
and 4 years.12

Some children with syndromic feet continue to relapse and treatment using the traditional
open soft-tissue releases as described above may be required. Occasionally the lateral column
will need to be shortened to correct severe adductus but, if the hindfoot remains in good
position, the adduction can be treated over the age of 6 years by a double tarsal wedge
osteotomy by transferring a wedge of bone from the cuboid to the medial column at the level
of the cuneiforms.12

Gradual correction using a circular external fixator (the Ilizarov method) has been reported in
treating difficult relapsed cases and severe deformities with good results. Full corrections can
be achieved even in feet severely scarred from previous surgery. The principles of Ponseti
can be adapted to use with the Taylor spatial frame (TSF) as elucidated by Herzenberg. The
procedure can be painful and long and is best reserved for these very difficult cases.12

Despite initially successful casting and subsequent surgery, some teratological deformities
may still recur. A deformed, stiff and painful foot in an adolescent is best salvaged by
corrective extra-articular osteotomies where possible and using tendon transfers to balance
the deforming forces. Arthrodesis is avoided if at all possible but in some cases this may be
the only viable salvage option available. The goal of all the treatment options is to finish with
a plantigrade, stable and pain-free foot by skeletal maturity.12

References
1. Kruse LM, Dobbs MB, Gurnett CA. Polygenic threshold model with sex dimorphism
in clubfoot inheritance: The Carter effect. J Bone Jt Surg - Ser A. 2008.
 doi:10.2106/JBJS.G.01346
2. Dobbs MB, Gurnett CA. Genetics of clubfoot. J Pediatr Orthop Part B. 2012.
doi:10.1097/BPB.0b013e328349927c
3. Faldini C, Prosperi L, Traina F, et al. Surgical treatment of neglected congenital
idiopathic talipes equinovarus after walking age in Eritrea: an Italo-Eritrean
cooperation. Musculoskelet Surg. 2016. doi:10.1007/s12306-016-0398-z
4. Owen RM, Kembhavi G. A critical review of interventions for clubfoot in low and
middle-income countries: Effectiveness and contextual influences. J Pediatr Orthop
Part B. 2012. doi:10.1097/BPB.0b013e3283499264
5. Chu A, Lehman WB. Treatment of Idiopathic Clubfoot in the Ponseti Era and Beyond.
Foot Ankle Clin. 2015. doi:10.1016/j.fcl.2015.08.002
6. Faldini C, Traina F, Nanni M, Sanzarello I, Borghi R, Perna F. Congenital idiopathic
talipes equinovarus before and after walking age: observations and strategy of
treatment from a series of 88 cases. J Orthop Traumatol. 2016. doi:10.1007/s10195-
015-0377-4
7. Parsa A, Moghadam MH, Taraz Jamshidi MH. Relapsing and residual clubfoot
deformities after the application of the Ponseti method: A contemporary review. Arch
Bone Jt Surg. 2014.
8. Carroll NC. Clubfoot in the twentieth century: Where we were and where we may be
going in the twenty-first century. J Pediatr Orthop Part B. 2012.
doi:10.1097/BPB.0b013e32834a99f2
9. Handelsman JE, Badalamente MA. Club foot: a neuromuscular disease. Dev Med
Child Neurol. 1982;24(1):3-12. doi:10.1111/j.1469-8749.1982.tb13577.x
10. Ippolito E, Maio F, Mancini F, Bellini D, Orefice A. Leg muscle atrophy in idiopathic
congenital clubfoot: Is it primitive or acquired? J Child Orthop. 2009.
doi:10.1007/s11832-009-0179-4
11. Chen C, Kaushal N, Scher DM, Doyle SM, Blanco JS, Dodwell ER. Clubfoot
Etiology: A Meta-Analysis and Systematic Review of Observational and Randomized
Trials. J Pediatr Orthop. 2018. doi:10.1097/BPO.0000000000001191
12. Blom A, Warwick D, Whitehouse M. Apley’s System of Orthopaedics and Trauma.
10th editi. Florida: CRC Press; 2018.
13. Zionts L. What’s New in Idiopathic Clubfoot?. J Pediatr Orthop. 2014.