Club Foot

Disease/Disorder

Definition

Clubfoot, or congenital talipes equinovarus, is a set of foot deformities of varying severity consisting of equinus (plantar flexion of talocrural joint), hindfoot varus (subtalar inversion), forefoot supination (adduction of talonavicular), and cavus of the midfoot. It is a significant cause of pain, disability, and deformity if left untreated.¹ 

Fig 1. Clubfoot deformity associated with equinus, hindfoot varus, midfoot cavus, and forefoot supination²

Etiology

The etiology of clubfoot is unknown but is likely multifactorial, with strong genetic factors along with poorly understood environmental factors.^1,3,4 In support of a genetic cause, results from twin studies showed concordance in monozygotic twins of 32% compared to dizygotic twins of 2.9%, as well as a familial recurrence of 10-20%.^3,4

Congenital clubfoot typically presents as an isolated defect, but can be seen in association with

• Myelomeningocele (spina bifida)
• Trisomy 18
• Ehlers-Danlos
• Larsen’s syndrome
• 22q11 deletion syndrome
• Distal arthrogryposis
• Congenital myotonic dystrophy

Epidemiology including risk factors and primary prevention

The incidence of clubfoot is 1-2/1000 live births throughout the world every year, but varies according to geographic location.¹ The deformity is bilateral in 50% of cases and is more common in males, with a 2:1 male to female ratio that remains consistent across ethnic populations. Chinese demonstrate the lowest incidence at 0.39 per 1000, and the highest incidence is found in Hawaiians and Māoris at 7 per 1000.^4,5 Currently, there is no known prevention. Intrauterine crowding was initially blamed, but has subsequently been disproven.¹ Smoking during pregnancy has been shown to be a significant and consistent environmental risk factor.^3,4

Patho-anatomy/physiology

Clubfoot is characterized not only by alignment issues, but also by changes in shape and size of the involved bones. The small sized foot and distorted talus are the most prominent features. Normally, the talus overlies the calcaneus. It is held within the ankle mortise and articulates anteriorly with the navicular. In clubfoot, the talus is smaller and externally rotated, causing a significant deformity. The navicular is displaced medially. These changes result in medial displacement and internal rotation of the talocalcaneonavicular joint. Soft tissue shortening in the posterior and medial compartments of the foot and ankle also occurs, which keeps the foot in adduction and equinovarus position.⁶

Fig 2. Pathological anatomy of clubfoot⁶

Clubfoot can have postural and structural characteristics that are classified by the Pirani and Dimeglio scales. These numerical scales apply a point-based valuation of physical characteristics of the foot that, when tallied, correlate with the severity of the deformity. They have been shown to be reliable and reproducible to assess the deformity, monitor progress of treatment, and identify early relapse.^5,7

Specific secondary or associated conditions and complications

Although patients demonstrate gait impairments, idiopathic clubfoot should not delay normal growth and development.⁸ Associated complications include chronic pain, stiffness, and skin lesions related to abnormal pressure and weight bearing on the lateral side of the foot.

Essentials of Assessment

History

Eighty percent of cases of clubfoot are idiopathic and found in otherwise healthy children.^1,5 Evaluation must include a comprehensive history focusing on signs and symptoms of associated conditions. Assessment should focus on the other limbs as well as skin abnormalities that can be predictive of neurological conditions. Birth history, development, and overall health status must be explored.

Physical examination

Physical examination must explore more than the foot/gait. Associated conditions such as neuromuscular or genetic disorders must be ruled out. ​A complete musculoskeletal exam, including assessment of active and passive range of motion of the foot and ankle, focal examination of the hips, and assessment of the spine for abnormality or dysraphism should occur. Other joints should be assessed for signs of abnormality or arthrogryposis, and the skin checked for cutaneous manifestations of spina bifida or other genetic diseases including hirsute patches, sacral dimples, and other mesodermal or limb anomalies.^1,5,6

Functional assessment

This should include

• Screening for developmental delays
• Assessment of mobility needs
• Assessing independence with self-care
• Evaluating effects on mood and behavior

Laboratory studies

Laboratory studies are not usually necessary. If neuromuscular disease or genetic syndromes are suspected, chromosome or genetic testing may assist in further diagnosis.

Imaging

Clubfoot may be detected during prenatal ultrasonography as early as 13 weeks’ gestation, though most often a prenatal diagnosis of clubfoot will occur at the routine ultrasound evaluation at 20 weeks’ gestation.^1,9 It is important to note that a mild isolated foot deformity on prenatal ultrasound may not be true clubfoot, as transient positional foot deformities are relatively common during pregnancy. The false positive rate of clubfoot on prenatal ultrasound is estimated to be approximately 20%.⁹ There is no prenatal treatment for clubfoot and diagnosis is confirmed by postnatal clinical examination of the foot.

In most cases, two view radiographs – anteroposterior and lateral – of the infant foot and ankle are obtained for baseline and to confirm appropriate correction after treatment.^1,6,10 Lines are drawn through the long axis of the talus and calcaneus to measure the talocalcaneal angle in both views. The normal angle is approximately 30-40˚. In clubfoot, these lines are almost parallel. A study by Sriharsha et al. found the Pirani and Dimeglio scores to correlate well with radiologic parameters, suggesting routine radiography can be avoided to prevent radiation exposure to infants with clubfeet.¹⁰

Supplemental assessment tools

Electrodiagnosis can be helpful in diagnosing associated neurological conditions.

Early predictions of outcomes

The Pirani and Dimeglio scales are used for classification, and higher scores correlate with worse outcomes.^5,7 In general, neurologically based clubfoot is typically more severe and resistant to treatment. It has a higher rate of relapse, especially using conservative treatment methods.^5,6

Environmental

Conservative clubfoot regimens require a minimum commitment of four years and are substantially impacted by poor compliance.^1,5 Education providing information and explaining expectations is important. Caring for a child in bilateral casts creates challenges. Transportation and access to providers can also be problematic, especially for patients living in rural areas.

Providers should address environmental barriers to treatment, including:

• Transportation
• Mobility
• Bathing/Hygiene
• Time – making appointments, performing stretching, bracing.

Rehabilitation Management and Treatments

Available or current treatment guidelines

Historically, management of clubfoot has fluctuated between conservative and surgical treatment. In 1948, treatment of clubfoot was revolutionized by Ignacio Ponseti at the University of Iowa. His treatment method was initially published in 1963 but not widely utilized until the 1990s. It establishes the most widely practiced treatment method for clubfoot regardless of etiology (idiopathic and non-idiopathic) and has significantly reduced the need for major foot surgery.^1,5,6 Studies reveal a correction rate of near 95% in idiopathic clubfoot.¹

At different disease stages

The goal of treatment is to establish a supple, anatomically aligned foot capable of functioning without causing pain, skin lesions, or disability.

Initial treatments are conservative and involve variations of stretching, splinting, and casting.^1,5,6 These take advantage of the pliability of an infant’s foot to gradually elongate the soft tissue and increase sarcomere length and number. Up to 37% of cases can relapse within two years, and this is noted mostly with non-compliance of splinting and stretching and/or progression of neuromuscular disease.^1,5

Treatment Methods

The Ponseti method is the treatment of choice today^1,5,6,11

• Long leg serial casting is used to obtain correction:
  • Order of correction is “CAVE” (cavus, adduction, varus, equinus);
  • Final cast positions the leg in 70 degrees of external rotation, 15-20 degrees of dorsiflexion; worn about three weeks.
• Patient is transitioned to a daytime AFO and a “boots and bar” foot abduction brace during sleep for four years.

Fig 3. Clubfoot brace with supramalleolar orthoses (boots) attached to a bar.

^{Image courtesy of Bassgrab75 under  CC BY-SA 4.0, via Wikimedia Commons}

The French functional method includes^1,5,6

• Combination of splinting, stretching, and taping;
• Initially done daily by physical therapist for the first 2 months, then frequency decreases to three times per week until 6 months of age, and finally transitioned to home exercise program until the child reaches walking age.

The Kite method differs subtly from Ponseti and entails

• Long leg casting for up to 2 years;
• 75% of cases require surgery due to incomplete corrections.^5,11

Achilles tenotomy is required in up to 90% of patients following casting. The patient’s age helps determine surgery technique.⁵

< 1 year old – percutaneous procedure/local anesthetic; usually performed in outpatient clinic.

> 1 year old – open procedure/general anesthesia typically used.

Although conservative treatment is often initially effective, Gelfer and colleagues suggest higher rates of recurrence and need for further surgery in children with non-idiopathic clubfoot. Recurrence occurs from muscular imbalance with over activity of tibialis anterior and poor evertor muscle function. Functional outcomes were improved by early surgical procedures to re-establish dorsiflexion and eversion.^12,13 Resistant cases can require further surgical care, including achilles tenotomy, tibialis anterior transfer, osteotomies and external fixation.⁶

Coordination of care

Each patient is unique and treatments must be individualized. Multidisciplinary care promoting communication between medical providers and caregivers is important. This contributes to improved functional outcomes and decreases complications from adverse events. Designated multidisciplinary clinics are optimal and allow for ease of communication among the providers on the treatment team.

Team members may include:

• Physiatrist and/or orthopedic surgeon​
• Physical therapist
• Orthotist
• Nursing educator

Patient & family education

Education of the family focuses on short- and long-term treatment expectations and goals. The importance of compliance with treatment regimens is stressed, as noncompliance is associated with increased treatment failure rates.^12,13

Emerging/unique interventions

Despite good initial correction of clubfoot deformity from the Ponseti method, relapse continues to challenge care of clubfoot. Newer studies suggest three-dimensional gait analysis as an objective assessment to evaluate functional outcome after treatment and to possibly identify early relapse. Grin et al. has shown patients with relapse of clubfoot have lower total gait quality and lower total foot scores, particularly in a newly introduced total foot score called the clubfoot deviation index, or cFDI.¹⁴

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Clubfoot extremities will always remain smaller than the comparative extremity and lateral skin creases will remain on the child’s foot, even after treatment is completed. Appropriately treated, most cases of idiopathic clubfoot will establish a plantigrade, pain-free, and functional foot, allowing the patient to participate in any activity they desire. Follow-up is required through the years of growth, and future intervention, including further surgical correction, may be necessary.^1,5,6,13

Cutting Edge/Emerging and Unique Concepts and Practice

Familial occurrence is well documented in clubfoot, prompting investigation into the genetic basis for non-syndromic clubfoot.^1,3,4 While several genetic variants have been shown to be associated with clubfoot, the mechanisms by which these genes confer risk is an area of ongoing research. Elucidation of the molecular pathophysiology of clubfoot may allow for accurate genetic counseling for at-risk families, development of prevention or screening programs, and improved management.¹⁵

Gaps in the Evidence-Based Knowledge

Surgery occurs anywhere from three months to one year of age. There are also some disagreements among treatment techniques, although conservative methods are generally favored. Efficacy of different treatments may be dependent on the stage in which they are employed (i.e., at birth vs. neglected presentation).¹¹

The certainty of the existing evidence regarding treatment outcomes in clubfoot remains low. A meta-analysis was attempted and the results were first published in the Cochrane Collaboration in 2010 and last updated in 2020. Twenty-one trials were available for evaluation, including 905 patients. While the formulation of strong conclusions was unsupported, this review did suggest that the Ponseti technique may produce better foot alignment in the short term with lower major surgery risk compared to the Kite technique in treatment-naïve cases. Furthermore, semi-rigid fiberglass may be as effective as plaster of Paris when using the Ponseti technique.¹¹

In the current body of evidence, adverse events have been reported inconsistently or altogether unreported, making comparisons difficult. Additionally, there has been limited use of validated outcome measures across trials. Well-powered, controlled trials are needed to better assess relevant treatment outcomes and determine efficacy.¹¹

References

1. Cady R, Hennessey T. Diagnosis and treatment of idiopathic congenital clubfoot. Pediatrics. 2022;149(2):e2021055555.
2. Gosse G, Ward E, McIntyre A, Banwell H. The reliability and validity of the weight-bearing lunge test in a Congenital Talipes Equinovarus population (CTEV). PeerJ. 2021;9:e10253.
3. Pavone V, Chisari E, Vescio A, Lucenti L, Sessa G, Testa G. The etiology of idiopathic congenital talipes equinovarus: a systematic review. J Orthop Surg Res. 2018;13:206.
4. Basit S, Khoshhal KI. Genetics of clubfoot; recent progress and future perspectives. European Journal of Medical Genetics. 2018;61(2):107-113.
5. Dobbs MB, Gurnett CA. Update on clubfoot: etiology and treatment. Clin Orthop. 2009;467(5):1146.
6. Hegazy M, Khairy H, Hegazy A, El-Aidy S. Clubfoot in children: an overview. Foot Ankle Online Journ. 2020;13(4):10.
7. Jain S, Ajmera A, Solanki M, Verma A. Interobserver variability in Pirani clubfoot severity scoring system between the orthopedic surgeons. Indian J Orthop. 2017;51(1):81-85.
8. Pavone V, Sapienza M, Vescio A, Caldaci A, McCracken K, Canavese F, Testa G. Early developmental milestones in patients with idiopathic clubfoot treated by Ponseti method. Front Pediatr. 2022;10:869401.
9. Faldini C, Fenga D, Sanzarello I, Nanni M, Traina F, Rosa M. Prenatal diagnosis of clubfoot: a review of current available methodology. Folia Medica. 2017;59(3):247-253.
10. Sriharsha Y, Balaji G, Bharathi D, Patro D. Do the clinical scores (Pirani and Dimeglio scores) correlate with the radiological parameters in idiopathic club foot in infants? A cross-sectional study. J Pediatr Orthop. 2021;30(5):471-477.
11. Bina S, Pacey V, Barnes EH, Burns J, Gray K. Interventions for congenital talipes equinovarus (clubfoot). Cochrane Database of Systematic Reviews. 2020(5). 
12. Gelfer Y, Dunkley M, Jackson D, Armstrong J, Rafter C, Parnell E, Eastwood D. Evertor muscle activity as a predictor of the midterm outcome following treatment of the idiopathic and non-idiopathic clubfoot. Bone Joint J. 2014;96:1264-1268.
13. Van Schelven H, Moerman S, Van Der Steen M, Besselaar A, Greve C. Prognostic factors for recurrent idiopathic clubfoot deformity: a systematic literature review and meta-analysis. Acta Orthop. 2022;93:11-28.
14. Grin L, Wijnands S, Besselaar A, Van Oorschot L, Vanwanseele B, Van Der Steen, M. The relation between clinical and objective gait scores in clubfoot patients with and without a relapse. Gait Posture. 2022;97:210-215.
15. Sadler B, Gurnett C, Dobbs M. The genetics of isolated and syndromic clubfoot. J Child Orthop. 2019;13(3):238-244.

Original Version of the Topic

Jane A. Emerson, MD. Club Foot, Published 3/14/2014

Previous Revision(s) of the Topic

Melissa Trovato, MD, William Ide, MD. Club Foot. 12/22/2020

Author Disclosures

Kavita Nadendla, MD
Nothing to Disclose

Melissa Trovato, MD
Nothing to Disclose