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.
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:
- Spina bifida
- Trisomy 18
- Larsen’s syndrome
- 22q11 deletion syndrome
- Distal arthrogryposis
- 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.
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 calcaneous. 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 occurs1,3,4.
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 assese the deformity and monitor progress 3,5.
Specific secondary or associated conditions and complications
Idiopathic clubfoot should not delay normal growth and development, although patients demonstrate gait impairments6. Associated complications include chronic pain, stiffness, and skin lesions related to abnormal pressure and weight bearing on the lateral side of the foot.
2. ESSENTIALS OF ASSESSMENT
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 heath status must be explored.
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 anomalies3,4,7.
This should include:
- Screening for developmental delays
- Assess mobility needs
- Assess independence with self-care
- Evaluate effects on mood and behavior
Laboratory studies are not usually necessary. If neuromuscular disease or genetic syndromes are suspected, chromosome or genetic testing may assist in further diagnosis1,7.
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 age 3,4,7. 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 topic4.
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 methods7,8.
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:
- Time – making appointments, doing stretching, bracing.
3. 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 surgery1,9,10. 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 casting1,2,11. 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 disease1,9.
A. The Ponseti Method is the treatment of choice today2,9,10,11.
- 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” brace during sleep for four years.
B. The French functional method includes:
- Combination of splinting, stretching, and taping;
- Initially done by physical therapist, then transitioned to home exercise program.
C. The Kite Method differs subtly from Ponseti and entails:
- Long leg casting for up to 2 years;
- 75% of cases require surgery3,12.
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 consistenty from muscular imbalance. Functional outcomes were improved by early surgical procedures to re-establish dorsiflexion and eversion13. 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:
- Physiatrist and/or orthopedic surgeon
- Physical therapist
- 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.
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 activity14.
Gait assessment in a formalized gait lab can also be a useful tool to provide objective information on the functional outcomes15.
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 necessary1,3,4.
4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE
5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE
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.
Botulinum toxin is sometimes utilized prior to casting or splinting and may play a role in treatment. Evidence for its use is currently lacking. Outcome data for clubfoot is currently limited. A recent meta-analysis was attempted and the results were published in the Cochrane Collaboration. Thirteen trials were available for evaluation, including 507 patients.
Outcome measures were so variable that meta-analysis was not possible. Authors also noted that most studies had no inclusion of adverse events, making comparisons difficult. Randomized trials are needed to better assess relevant treatment outcomes and determine efficacy2.
1. Alexander M, Ackman, JD, Kuo, KN. Congenital idiopathic clubfoot. Orthopedic Nursing 1999;18:47-57.
2. Gray K, Pacey, V, Gibbons, P, et al. Interventions for congenital talipes equinovarus (clubfoot). Cochrane Database Syst Rev 2012 April 18;4:CD008602.doi.1002/14651858.CD008602.pub2.
3. Dobbs MB, Gurnett, CA. Update on club foot: etiology and treatment. Clin Orthop Relat Res 2009;467:1146-1153.
4. Gobbins PJ, Kelly G. Update on club foot. J Paediatr Child Health. 2013;49:E434-E437.
5. Wainwright, AM Auld, T, Benson, MK, et al. The classification of congenital talipes equinovarus. J Bone Joint Surg [Br] 2002;84-B:1020-1024.
6. Sala, DA, Chu, A, Lehman, WB, et al. Achievement of gross motor milestones in children with idiopathic club foot treated with the Ponseti method. J Pediatr Orthop 2013;33:55-58.
7. Dobbs, MB, Gurnett, CA. Genetics of clubfoot. J Paediatr Orthop B 2012;21:7-9.
8. Gurnett, CA, Boehm, S, Connolly, A, et al. Impact of congenital talipes equinovarus etiology on treatment outcomes. Dev Med Child Neurol 2008;50:498-502.
9. van Bosse, H. Ponseti treatment for clubfeet: an international perspective. Current Opinion in Pediatrics 2011;23:41-45.
10. Zionts, LE, Sangiorgio, SN, Ebramzadeh, E, et al. The current management of idoppathic clubfoot revisited: results of a survey of the POSNA membership. J Pediatr Orthop 2012;32:515-520.
11. Scher DM, Feldman, DS, van Boss HJ, et al. Predicting the need for tenotomy in the Ponseti method for correction of clubfeet. J Pediatr Orthop 2004;24:349-352.
12. Faulks S, Richards, SB. Clubfoot treatment: Ponseti and French functional methods are equally effective. Clin Orthop Relat Res 2009;467:1278-1282.
13. Roux, A, Laville, JM, Rampal, V, et al. Paralytic congenital talipes equinovarus of unknown origin: a new entity. Multicenter study of 42 cases. Orthop Trumatol Surg Res 2012;98:570-575.
14. Andriesse, H, Hagglund, G, Jarmio, G-B, et al. The clubfoot assessment protocol (CAP): description and reliability of a structured multi-level instrument of follow-up. BMC Musculoskeletal Disord 2005;6:40 doi: 10.1186/1471-2474-6-40 [Date accessed 12-17-13].
15. Karol LA, Jeans, KA. Assessment of clubfoot treatment using movement analysis. J Exp Clin Med 2011;3:228-232.
Jane A. Emerson, MD
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