Author(s): Melissa Trovato, MD, William Ide, MD
Originally published: March 14, 2014 Last updated: December 22, 2020
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Disease/ Disorder

Definition

Clubfoot, or congenital talipes equinovarus (CTEV) is a set of foot deformities of varying severity, consisting of equinus (plantar flexion of talocrual 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 untreated1,2.

Etiology

The etiology of clubfoot is unknown, but is likely multifactorial. The incidence in first-degree relatives is increased at 2-3%, and the concordance among identical twins is 33%1. Nearly 25% of cases are familial3.

It presents commonly as an isolated defect but can be seen in association with:

  1. Spina bifida
  2. Trisomy 18
  3. Ehlers-Danlos
  4. Larsen’s syndrome
  5. 22q11 deletion syndrome
  6. Distal arthrogryposis
  7. Congenital myotonic dystrophy

Epidemiology including risk factors and primary prevention

The incidence of clubfoot is 1-1.2/1000 live births in the United States and varies according to geographic location. 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 Maoris at 7 per 1000. Currently there is no known prevention. Intrauterine crowding was initially blamed, but has subsequently been disproven1,3.  Smoking during pregnancy has been shown to be a significant and consistent environmental risk factor4.

Patho-anatomy/physiology

Clubfoot is characterized not only by alignment issues, but also by changes in shape and size of the involved bones. The deformity causes changes at the talocalcaneonavicular joint which is displaced medially and internally rotated. The talus normally overlies the calcaneus. It is held within the ankle mortise and articulates anteriorly with the navicular. In clubfoot, it is smaller and externally rotated, causing a significant deformity. The navicular is displaced medially. Soft tissue shortening in the posterior and medial compartments of the foot and ankle also occurs1,3,5.

Clubfoot can have postural and structural characteristics that are classified by the Pirani and Demeglio 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 and monitor progress3,6.

Specific secondary or associated conditions and complications

Idiopathic clubfoot should not delay normal growth and development, although patients demonstrate gait impairments7. 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 clubfeet are idiopathic and found in otherwise healthy children3. 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 assessments 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 hirsuit patches, sacral dimples, and other mesodermal or limb anomalies3,5,8.

Functional assessment

This should include:​

  1. Screening for developmental delays
  2. Assess mobility needs
  3. Assess independence with self-care
  4. Evaluate 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 diagnosis1,8.

Imaging

In most cases, two view radiographs of the foot and ankle are obtained. Ultrasound remains the modality of choice in infants less than 3 months of age3,5,8. Lateral x-rays further delineate the deformity. Lines are drawn through the long axis of the talus and calcaneus. These should intersect at a 30-40 degree angle. If they are parallel, surgery is required. On the anterior/posterior view, the talus and calcaneus should be divergent. If they overlap, this is indicative of poor alignment.

Prenatal screening is easily accomplished by ultrasound, but universal screening remains a debatable topic5.

Supplemental assessment tools

Electrodiagnosis can be helpful in diagnosing associated neurological conditions.

Early predictions of outcomes

The Pirani and Demeglio scales are used for classification, and higher scores correlate with worse outcomes3. In general, neurologically based clubfoot is typically more severe and resistant to treatment. It has a higher rate of relapse, especially using conservative treatment methods8,9.

Environmental

Conservative clubfoot regimens require a minimum commitment of four years and are substantially impacted by poor compliance. 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:

  1. Transportation
  2. Mobility
  3. Bathing/Hygiene
  4. Time – making appointments, doing stretching, bracing.

Rehabilitation Management and Treatments

Available or current treatment guidelines

Treatments were revolutionized by Ponseti at the University of Iowa in 1948. His treatment method was initially published in 1963 but not widely utilized until the 1990s. It establishes the most widely practiced treatment method and has significantly reduced the need for major foot surgery1,10,11. Studies reveal a correction rate of near 95% in idiopathic clubfoot1.

There are no established treatment guidelines.

At different disease stages

The aim 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 casting1,2,12. These take advantage of the pliability of an infant’s foot and 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 and/or progression of neuromuscular disease1,10.

Treatment Methods:

A. The Ponseti Method is the treatment of choice today2,10-12.

  1. 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.
  2. Patient is transitioned to a daytime AFO and a “boots and bar” brace during sleep for four years.

B. The French functional method includes:

  1. Combination of splinting, stretching, and taping;
  2. Initially done by physical therapist, then transitioned to home exercise program.

C. The Kite Method differs subtly from Ponseti and entails:

  1. Long leg casting for up to 2 years;
  2. 75% of cases require surgery3,13.

Achilles tenotomy is required in up to 90% of patients following casting. The patient’s age helps determine surgery technique3:

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

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

Roux and colleagues suggested that early surgical correction was indicated over conservative techniques in patients with neuromuscular paralysis. Although conservative treatment was initially effective, recurrence occurs consistently from muscular imbalance. Functional outcomes were improved by early surgical procedures to re-establish dorsiflexion and eversion14.Resistant cases can require further surgical care, including achilles tenotomy, tibialis anterior transfer, osteotomies and external fixation. Dobbs and colleagues suggested that poor long-term results are noted with extensive soft-tissue release surgery3.

Environmental modifications may be necessary to assist mobility and ensure optimal safety and function.

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:

  1. Physiatrist and/or orthopedic surgeon​
  2. Physical therapist
  3. Orthotist
  4. 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.

Emerging/Unique Interventions

The clubfoot assessment protocol is a multi-dimensional, observer-administered instrument, which longitudinally assesses clubfoot deformity through growth. It was created from 22 items and subdivided by body function, structure, and activity15.

Gait assessment in a formalized gait lab can also be a useful tool to provide objective information on functional outcomes16.

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 necessary1,3,5.

Cutting Edge/ Emerging and Unique Concepts and Practice

Familial occurrence and higher concordance in monozygotic twins compared to dizygotic twins has prompted investigation into the genetic basis for non-syndromic clubfoot4. 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 and improved management4.

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)17.

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 technique17. 

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 efficacy2,17.

References

  1. Alexander M, Ackman JD, Kuo KN. Congenital idiopathic clubfoot. Orthopaedic Nursing. 1999;18(4):47.
  2. Gray K, Pacey V, Gibbons P, Little D, Burns J. Interventions for congenital talipes equinovarus (clubfoot). Cochrane database of systematic reviews. 2014(8).
  3. Dobbs MB, Gurnett CA. Update on clubfoot: Etiology and treatment. Clin Orthop. 2009;467(5):1146.
  4. Basit S, Khoshhal KI. Genetics of clubfoot; recent progress and future perspectives. European Journal of Medical Genetics. 2018;61(2):107-113.
  5. Gibbons PJ, Gray K. Update on clubfoot. J Paediatr Child Health. 2013;49(9):E434-E437.
  6. Wainwright AM, Auld T, Benson MK, Theologis TN. The classification of congenital talipes equinovarus. The Journal of Bone and Joint Surgery.British volume. 2002;84(7):1020-1024.
  7. Sala DA, Chu A, Lehman WB, van Bosse HJ. Achievement of gross motor milestones in children with idiopathic clubfoot treated with the ponseti method. Journal of Pediatric Orthopaedics. 2013;33(1):55-58.
  8. Dobbs MB, Gurnett CA. Genetics of clubfoot. Journal of pediatric orthopaedics.Part B. 2012;21(1):7.
  9. Gurnett CA, Boehm S, Connolly A, Reimschisel T, Dobbs MB. Impact of congenital talipes equinovarus etiology on treatment outcomes. Developmental Medicine & Child Neurology. 2008;50(7):498-502.
  10. van Bosse HJ. Ponseti treatment for clubfeet: An international perspective. Curr Opin Pediatr. 2011;23(1):41-45.
  11. Zionts LE, Sangiorgio SN, Ebramzadeh E, Morcuende JA. The current management of idiopathic clubfoot revisited: Results of a survey of the POSNA membership. Journal of Pediatric Orthopaedics. 2012;32(5):515-520.
  12. Scher DM, Feldman DS, van Bosse HJ, Sala DA, Lehman WB. Predicting the need for tenotomy in the ponseti method for correction of clubfeet. Journal of Pediatric Orthopaedics. 2004;24(4):349-352.
  13. Faulks S, Richards BS. Clubfoot treatment: Ponseti and french functional methods are equally effective. Clin Orthop. 2009;467(5):1278.
  14. Roux A, Laville JM, Rampal V, Seringe R, Salmeron F. Paralytic congenital talipes equinovarus of unknown origin: A new entity. multicenter study of 42 cases. Orthopaedics & Traumatology: Surgery & Research. 2012;98(5):570-575.
  15. Andriesse H, Hägglund G, Jarnlo G. The clubfoot assessment protocol (CAP); description and reliability of a structured multi-level instrument for follow-up. BMC Musculoskeletal Disorders. 2005;6(1):40.
  16. Karol LA, Jeans KA. Assessment of clubfoot treatment using movement analysis. Journal of Experimental & Clinical Medicine. 2011;3(5):228-232.
  17. Bina S, Pacey V, Barnes EH, Burns J, Gray K. Interventions for congenital talipes equinovarus (clubfoot). Cochrane Database of Systematic Reviews. 2020(5). 

Original Version of the Topic

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

Author Disclosures

Melissa Trovato, MD
Nothing to Disclose

William Ide, MD
Nothing to Disclose