Talipes equinovarus (TEV), commonly known as congenital clubfoot, is one of the most common congenital musculoskeletal deformities, occurring in approximately 1 in every 1,000 live births. It is a complex three-dimensional deformity of the foot characterized by cavus, forefoot adduction, hindfoot varus, and ankle equinus (CAVE). The deformity is usually idiopathic but may also occur as part of neuromuscular or syndromic disorders.

Without treatment, children develop progressive foot deformity, abnormal gait, pain, callosities, recurrent skin ulceration, and lifelong disability. Early diagnosis and treatment using the Ponseti method achieve excellent functional outcomes in more than 90% of cases.

Epidemiology

• Incidence approximately 1 per 1,000 live births

• Male-to-female ratio approximately 2:1

• Bilateral involvement in about 50% of patients

• More common in individuals with a positive family history

• Idiopathic clubfoot accounts for approximately 80% of cases

Etiology

The exact cause remains unknown in most patients. Clubfoot is considered a multifactorial developmental disorder involving genetic and environmental factors.

Idiopathic

• Most common form

• Isolated congenital deformity

Syndromic or secondary clubfoot

Associated with:

• Spina bifida

• Arthrogryposis multiplex congenita

• Congenital myotonic dystrophy

• Diastrophic dysplasia

• Larsen syndrome

• Neuromuscular disorders

Pathophysiology

Congenital clubfoot results from abnormal fetal development of the muscles, tendons, ligaments, and bones of the foot and ankle during early gestation. The affected soft tissues, particularly the Achilles tendon, posterior tibial tendon, joint capsules, and medial ligaments, become shortened and contracted. These contractures pull the developing foot into plantarflexion, inversion, and medial rotation.

Internally, the talus is abnormally shaped and medially rotated, while the navicular and calcaneus are displaced medially around the talar head. These structural abnormalities produce the characteristic combination of cavus, forefoot adduction, hindfoot varus, and equinus deformities. Without early correction, progressive fibrosis of the soft tissues and adaptive bony remodeling make the deformity increasingly rigid and difficult to treat.

Risk factors

• Positive family history

• Male sex

• Oligohydramnios

• Maternal smoking during pregnancy

• Neuromuscular disorders

• Syndromic conditions

• Multiple gestation

• Congenital anomalies

Classification

According to etiology

Idiopathic clubfoot

• Most common

• Isolated deformity

Syndromic clubfoot

Associated with congenital disorders.

Neurogenic clubfoot

Occurs with neurological abnormalities such as spina bifida.

According to severity

Severity may be assessed using validated scoring systems including:

• Pirani score

• Dimeglio classification

These scores guide treatment and monitor correction.

Clinical presentation

The deformity is present at birth.

Characteristic foot deformities (CAVE)

• Cavus (high medial longitudinal arch due to tight intrinsic muscles)

• Forefoot adduction (tight tibialis posterior)

• Hindfoot varus

• Equinus (tight Achilles tendon)

Physical findings

• Small foot

• Small calf muscles

• Deep medial plantar crease

• Posterior heel crease

• Rigid inward-turned foot

• Limited ankle dorsiflexion

• Inability to place sole flat on the ground

In untreated older children:

• Toe walking

• Lateral border weight-bearing

• Callosities

• Shoe wear difficulties

• Progressive disability

Diagnostic criteria

Diagnosis is primarily clinical.

Diagnosis is established by:

• Presence of all four components of the CAVE deformity

• Rigid deformity that cannot be fully corrected by passive manipulation

• Absence or presence of associated syndromic features

• Severity assessed using the Pirani or Dimeglio scoring systems

Prenatal diagnosis may be suspected by obstetric ultrasonography during the second trimester.

Investigations

Clinical assessment

• Complete musculoskeletal examination

• Assessment for associated congenital anomalies

• Hip examination

• Spine examination

• Neurological assessment

Imaging

Routine imaging is not required in typical idiopathic clubfoot.

Plain radiographs may be useful:

• In atypical cases

• Older untreated children

• Recurrent deformity

• Surgical planning

Prenatal ultrasonography may identify clubfoot before birth.

Differential diagnosis

• Positional foot deformity

• Metatarsus adductus

• Congenital vertical talus

• Calcaneovalgus foot

• Neuromuscular foot deformities

• Arthrogryposis

Management

Early treatment should ideally begin within the first 1–2 weeks after birth.

Non-operative management (Ponseti method)

The Ponseti method is the gold standard treatment worldwide.

The goal is to rotate the foot laterally around a fixed talus while correcting deformities in the following sequence (CAVE):

1. Cavus

2. Adductus

3. Varus

4. Equinus

Stage 1: Serial manipulation and casting (Birth to approximately 2 months)

Weekly manipulation followed by long-leg plaster casting with the knee flexed to approximately 90°.

First correction

Correct cavus by:

• Supinating the forefoot

• Aligning the forefoot with the hindfoot

Pronation should never be used, as it worsens cavus.

Second correction

Correct:

• Forefoot adduction

• Heel varus

By abducting the foot around the head of the talus, which acts as the fulcrum.

Achilles tenotomy

Percutaneous Achilles tendon tenotomy is required in 80–90% of children.

Perform when:

• Foot abduction reaches at least 60°

• Heel is in valgus

• Residual equinus persists

Following tenotomy:

• Apply a long-leg cast in maximum dorsiflexion

• Maintain for 3 weeks

Stage 2: Foot abduction orthosis (approximately 4–8 months)

Following correction:

Wear the foot abduction orthosis:

• 23 hours per day for the first 3 months

Then

• During sleep and naps until approximately 4 years of age

Brace position:

• Affected foot: at least 60° external rotation

• Normal foot (unilateral cases): approximately 30° external rotation

The brace should be measured before tenotomy so it is available immediately after cast removal.

Failure to comply with brace wear is the most important risk factor for recurrence.

Stage 3: Management of recurrence (2–5 years)

Approximately 30–50% of children may require:

Tibialis anterior tendon transfer, with or without:

• Repeat Achilles tenotomy

• Gastrocnemius recession

Indications include:

• Dynamic forefoot supination during gait

• Recurrent deformity despite appropriate bracing

Operative management

Surgery is reserved for resistant, neglected, syndromic, or recurrent deformities when Ponseti treatment fails.

Children younger than 2 years

• Posteromedial soft tissue release

• Tendon lengthening procedures

Children older than 2 years

Depending on deformity:

• Medial column lengthening

• Lateral column shortening osteotomy

• Cuboid decancellation osteotomy

Severe rigid recurrent deformity (6–10 years)

Talectomy may be considered in:

• Severe rigid recurrent clubfoot

• Arthrogryposis-associated deformity

Postoperative treatment should always be complemented with:

• Serial casting

• Foot abduction orthosis

Complications

Untreated disease

• Permanent foot deformity

• Abnormal gait

• Pain

• Callosities

• Skin ulceration

• Functional disability

Treatment-related complications

• Recurrence

• Overcorrection

• Undercorrection

• Cast sores

• Infection after surgery

• Stiff foot

• Residual muscle weakness

Prognosis

When treatment begins shortly after birth and the Ponseti protocol is followed correctly, more than 90–95% of children achieve a painless, plantigrade, flexible, and functional foot without the need for extensive surgery. The prognosis is strongly influenced by adherence to the foot abduction orthosis, as poor brace compliance remains the leading cause of recurrence.

Prevention

There is no known method of preventing idiopathic clubfoot. Early recognition, prompt referral to a Ponseti-trained provider, and strict adherence to brace treatment substantially reduce recurrence and improve long-term functional outcomes.

Patient education

Parents and caregivers should be advised to:

• Begin treatment as early as possible after birth.

• Attend all scheduled casting appointments.

• Understand that Achilles tenotomy is commonly required and is part of standard treatment.

• Ensure strict compliance with the foot abduction orthosis.

• Inspect the child's skin regularly for pressure sores.

• Return promptly if the foot begins turning inward again.

• Continue follow-up until skeletal maturity because recurrence can occur during childhood.