Congenital Talipes Equinovarus

Definition of the disease

A clubfoot, Giles Smith syndrome [1]

or talipes equinovarus (TEV), is a

birth defect. TEV is classified into 2 groups: Postural TEV or Structural TEV.

Without treatment, persons afflicted often appear to walk on their ankles, or

on the sides of their feet. It is a common birth defect, occurring in about one

in every 1,000 live births. Approximately 50% of cases of clubfoot are

bilateral. In most cases it is an isolated dysmelia. This occurs in males more

often than in females by a ratio of 2:1.

Clubfoot is a congenital foot abnormality. It is one of the most common

pediatric orthopedic conditions. The heel tilts in and down, and the forefoot

is also turned in. Without treatment,a patient with a clubfoot will walk on the

outside of the foot which may produce pain and/or disability. A clubfoot is

usually smaller than an unaffected (normal) foot.

TEV is associated with joint laxity, congenital dislocation of the hip,

tibial torsion, ray anomalies of the foot (oligodactyly), absences of some

tarsal bones and a history of other foot anomalies in the family

Etiology

The exact cause of the clubfoot is unknown. It cannot be prevented,

but it is treatable. A child with a clubfoot should still grow and develop

normally and be able to walk, run and play sports.

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 While there is no conclusive proof that talipes is hereditary, statistics

indicate that there is a three to four percent probability that a child will

develop it if one parent has this deformity. This percentage increases to 15

percent if both parents are affected. For normal parents who have a child

with this problem, there is a two to five percent chance that their next child

will have talipes. Additionally, male babies are twice as likely to be afflicted

with this condition than female babies.

Structural TEV is caused by: genetic factors, a genetic defect with

three copies of chromosome 18. Growth arrests at roughly 9 weeks and

compartment syndrome of the affect limb are also causes of Structural TEV.

Genetic influences increase dramatically with family history. It was

previously assumed that postural TEV could be caused by external influences

in the final trimester such as intrauterine compression from oligohydramnios

or from amniotic band syndrome. However, this is countered by findings that

TEV does not occur more frequently than usual when the intrauterine space

is restricted.

The cause of talipes is the abnormal position of the feet and limited

space in the womb during fetal development, which can result in malformed

tendons, muscles and bones. Furthermore, the lack of amniotic fluid in the

sac surrounding the fetus, or oligohydramnios, can increase pressure on the

feet, leading to talipes as well.

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Clinical Manifestations

Clinical manifestation observed in each patients included the following:

contracture of the heel-cord triceps surae; contracture of the posterior tibial,

flexor hallucis longus, and flexor digitorum longus tendons: contracture of

the posterior capsule including the tibiotalar and fibulotalar articulations (the

posterior talocalcaneal joint was not opened); contracture of the deltoid

ligament, involvement of the anterior tibiotalar ligament because of the

forward subluxation of the talus; fixation of the scaphoid to the medial

malleolus with contracture of the talonavicular and talocalcaneal ligaments;

the anterior tibial tendon was moderately shortened, and the cuboid was

displaced medially and the calcaneocuboid joint was moderately distorted.

The combination of navicular displacement and medial and posterior ankle

and foot contractures was associated with internal rotation of the talus in the

ankle mortise and the calcaneus with the talus.

Pathophysiology

Environment Genetic factors Uterine position

factors
Chromosome 18
smoking

9-12 weeks to
gestation

Development of
3 Lower limbs are
Lower limbs are
misrepresented deformed
 Genetic and ecological factors are important in the pahophysiology of

TEV. There is substantiation that maturity of bone, joint, connective tissue,

innervation, vasculature and muscle may each be implicated in the

pathophysiology. annoyance of the overall process of medial rotation of the

fetal foot may be the common pathway linked to all these aspects of

development. It is likely there is more than one different reason, and at least

in some cases the phenotype may occur as a result of a threshold effect of

different factors acting together.

Pathology observed in each of the three feet at operation included the

following: (1) contracture of the heel-cord triceps surae; (2) contracture of

the posterior tibial, flexor hallucis longus, and flexor digitorum longus

tendons: (3) contracture of the posterior capsule including the tibiotalar and

fibulotalar articulations (the posterior talocalcaneal joint was not opened); (4)

contracture of the deltoid ligament, involvement of the anterior tibiotalar

ligament because of the forward subluxation of the talus; (5) fixation of the

scaphoid to the medial malleolus with contracture of the talonavicular and

talocalcaneal ligaments; (6) the anterior tibial tendon was moderately

shortened, and (7) the cuboid was displaced medially and the

calcaneocuboid joint was moderately distorted. (8) The combination of

navicular displacement and medial and posterior ankle and foot contractures

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was associated with internal rotation of the talus in the ankle mortise and the

calcaneus with the talus.

The articular surface of the head of the talus was covered medially by

the scaphoid and the body of the talus was turned inward as far as the

medial border of the talus would allow. A portion of the head of the talus was

uncovered laterally. Paradoxically, it appeared to point laterally but actually

was pointing medially in relation to the ankle joint. Indentation of the medial

malleolus on the body of the talus is readily visualized once the ligaments

have been released.

When the talus and calcaneus are derotated laterally as a unit, the

area of medial compression on the body of the talus due to inversion and

internal rotation becomes evident, and when these conditions are corrected

a space appears between the talus and the medial articular surface of the

medial malleolus.

Diagnostic Test

1. X- Ray

2. Magnetic Resonance Imaging

Medical Intervention

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There are two well-known non-surgical methods to treat talipes:

a) The Ponseti method

A special splint called the Denis-Browne splint is put in place. This

splint is made of two high-top, open-toed shoes connected by a bar, which is

adjusted according to the correct position of the feet. The splint is worn full-

time for two to three months and then only at night for the next two to four

years.

Developed by Dr. Ignacio Ponseti of Iowa, the Ponseti method begins

with a series of gentle manipulations and toe-to-thigh cast placements for

the first five to seven weeks. The heel-cord is cut to complete the correction

of the foot before the last cast is applied. Then, the last cast is placed for

three weeks, by which time the heel-cord has healed properly.

b) French Method

This treatment requires daily one-hour therapy with a qualified

physical therapist. It consists of gentle stretching of the feet, followed by

taping to maintain their improved position. At night, the taped feet are

connected with a machine that allows for a continuous passive motion to

maximize stretching. For two hours every day, the tape is removed to air the

skin. Physical therapy sessions continue daily for as long as three months,

and taping is discontinued once the child begins walking.

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 In stretching and casting therapy the doctor changes the cast multiple

times over a few weeks, gradually stretching tendons until the foot is in the

correct position of external rotation. The heel cord is released (percutaneous

tenotomy) and another cast is put on, which is removed after three weeks.

To avoid relapse a corrective brace is worn for a gradually reducing time

until it is only at night up to four years of age.

Surgical Intervention

The operative procedure includes: (1) Z-lengthening of the heel cord

with release of the medial fibers distally; (2) capsulorrhaphy of the tibiotalar

and fibulotalar joints complete; (3) capsulorrhaphy of the deltoid ligament

and plantar calcaneonavicular ligament maintaining a small tongue of

capsule attached to the tibia; (4) capsulorrhaphy of the talonavicular

ligaments: (5) capsulorrhaphy of the anterior tibiotalar ligament from medial

to lateral malleolus. (The talocalcaneal ligament and the posterior

compartment fo the foot should be avoided.) (6) The talus is then rotated

laterally in the ankle mortise and the calcaneus with it. If derotation is not

complete and if the scaphoid does not glide readily to the lateral side, a

second incision is made laterally, opening the calcaneocuboid joint and the

cuboid metatarsal joints. The sinus tarsi is entered and the lateral

talonavicular and calcaneonavicular ligaments are released, as are the

tibulotalar ligaments laterally.

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 This procedure usually allows full external rotation of the talus and the

calcaneus as a unit and reestablishes the lateral border of the foot - the

calcaneocuboid angle being changed from convex to neutral. The

relationship between the talus and the calcaneus is reestablished and the

foot is then lined up with the fibula and medial malleolus. (7) The anterior

tibial tendon is detached from the first metatarsal on the medial side and

transferred to the dorsum of the first metatarsal where it is reinserted into

soft tissue and periosteum in the infant, or into a hole in the first cuneiform

in the older patient.

All tendons that have been lengthened are resutured and none is

resected. The posterior tibial tendon should be saved and every effort should

be made to maintain its original action on the hindfoot. At this point in the

operation, excessive correction or the creation of heel valgus is readily

possible, particularly in the foot of a child under one year of age. Resuture of

a portion of the deltoid ligament and the medial tendons will avoid excessive

correction and allow active forces to be applied to the medial border of the

foot as the child grows.

The tourniquet is released, the bleeding points are coagulated, and the

skin is closed with absorbable suture. In the infant, fixation pins are usually

unnecessary to maintain the relationship between the scaphoid and talus but

may he required in the older child. Excessive abduction of the forefoot

should be avoided and the relationship between the talus and the scaphoid,

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and the talus and ankle joint, should approach that seen in the physiologic

position. If the skin is tight after closure, the foot should be inverted slightly

and the tension taken off the skin.

Primary healing should be sought. Fluffed gauze and pads are applied,

sheet cotton is used and split so that there is no pressure on the skin, and

the cast is applied with the foot at right angles and in neutral position in

relationship to the malleoli, with the knee at right angles. This cast is

changed at 10 to 14 day's so that repositioning can be accomplished if there

was tension on the skin initially. At this time the initial internal rotation of the

foot and the apparent, but usually nonexistent, tibial torsion have been

corrected. In over 90 percent of the clubfeet treated, the internally rotated

position has been corrected by derotating the talus in the ankle mortise. In

the author's opinion the ligamentous contracture, the position of the talus in

the ankle mortise, and the relationship of the adjacent tarsal bones to the

talus constitute the primary pathology in congenital talipesequinovarus. If all

aspects of the deformity are not corrected, then recurrent clubfoot is the

rule.

Operative correction must be made from both the medial and lateral

sides of the ankle joint and the midfoot in order to fully' correct the internal

rotation of the talus and the medial positioning of the scaphoid, as well as

the distortion of the calcaneocuboid joint.

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 Any procedure that attempts to do this without correcting the talus

may be successful only because the total deformity was very mild. Severe

clubfeet will not be corrected permanently if the ankle joint is not released.

Total subtalar release will not be successful in the severe foot and will result

eventually in a distorted tibiotalar joint with a foot that is apparently

corrected by external rotation but which eventually will either revert to varus

or will be placed in a valgus position in overcorrection.

Nursing Intervention

This treatment for talipes require nursing care which consists of gentle

manipulations to move the feet as much as possible towards the correct

position and to hold this correction with a plaster cast. Splints are then used

as a follow-up treatment. These are flexible, as they can be used at various

times during the treatment and can be worn 24 hours a day or only at night.

Shoes used with splints should be straight laced shoes with a straight medial

border.

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