At a glance......
- 1 Pathoanatomy of Clubfoot
- 2 Types of Clubfoot
- 3 Causes of Clubfoot
- 3.1 Genetics
- 3.2 Mechanical Factors in Utero
- 3.3 Neuromuscular Defect
- 3.4 Primary Germ Plasma Defect
- 3.5 Arrested Fetal Development
- 3.6 Hereditary
- 3.7 The Mechanical Forces or Positional Hypothesis
- 3.8 The Bone/Joint Hypothesis
- 3.9 The Connective Tissue Hypothesis
- 3.10 The Vascular Hypothesis
- 3.11 Support for a Neurological Hypothesis
- 3.12 The Developmental Arrest Hypothesis
- 3.13 Others
- 4 Symptoms of Clubfoot
- 5 Diagnosis of Clubfoot
- 6 Treatment of Clubfoot
- 7 Complications
Testing / Diagnosis for Clubfoot in Children/Clubfoot (congenital talipes equinovarus) also referred to as clubfoot is one of the most common birth defects involving the musculoskeletal system. Although clubfoot is recognizable at birth, the severity of the deformity can vary from mild to an extremely rigid foot that is resistant to manipulation. Two classification systems are widely used in the initial evaluation of clubfoot deformities. It is defined as fixation of the foot in adduction, in supination and in varus, i.e. inclined inwards, axially rotated outwards and pointing downwards [rx]. The calcaneus, navicular and cuboid bones are medially rotated in relation to the talus, and are held in adduction and inversion by ligaments and tendons.
Clubfoot is a birth defect where one or both feet are rotated inward and downward.[rx][rx] The affected foot and leg may be smaller than the other.[rx] In about half of those affected, both feet are involved.[rx] Most cases are not associated with other problems.[rx] Without treatment, people walk on the sides of their feet, which causes problems with walking.[rx]
Pathoanatomy of Clubfoot
Numerous anatomical studies of clubfoot have confirmed the gross changes in the shape and position of the talus, navicular, calcaneus and cuboid [rx,rx]. The tendons, tendon sheaths, ligaments, and fascia of the foot have undergone adaptive changes and became fibrotic or contractured [rx, rx]. The talocalcaneocuboid joints are subluxated [rx], [rx], [rx], [rx]. Nevertheless, until today, the question still remains as to whether the initial anatomical changes first occurred in the tarsal bones with subsequent soft tissue adaptation or vice versa.
Types of Clubfoot
The purpose of a classification system is to help in subsequent management and prognosis. Various classifications of clubfoot exist in the literature [rx, rx, rx,rx]. However, without a uniform standard, these classifications pose a major problem. Furthermore, some are too complex for practical use.
Dimeglio in 1991 divided clubfeet into 4 categories based on joint motion and ability to reduce the deformities [rx].
- Soft foot – may also be called postural foot and corrected by standard casting or physiotherapy treatment.
- Soft > Stiff foot – 33% of cases. It is usually a long foot which is more than 50% reducible and responds initially to casting. However, if the total correction has not been achieved after 7 or 8 months, surgery must be performed.
- Stiff > Soft foot – 61% of cases. It is less than 50% reducible and after casting or physiotherapy, it is released surgically according to specific requirements,
- Stiff foot – it is teratologic and poorly reducible. It is in severe equinus deformity, often bilateral and requires an extensive surgical correction.
This serves two purposes
- Completely correcting the clubfoot – as the definitive treatment. Mild clubfoot may fall into this category.
- Partially correcting a rigid clubfoot thereby – making the surgical approach less extensive [rx, rx]. Casting tends to prevent further tightening of the contracted structures during the interval prior to surgery [rx].
Clubfoot is often broadly classified into two major groups
- Isolated (idiopathic) clubfoot – is the most common form of the deformity and occurs in children who have no other medical problems.
- A nonisolated clubfoot – occurs in combination with various health conditions or neuromuscular disorders, such as arthrogryposis and spina bifida. If your child’s clubfoot is associated with a neuromuscular condition, the clubfoot may be more resistant to treatment, require a longer course of nonsurgical treatment, or even multiple surgeries.
Causes of Clubfoot
The primary causes of the deformity. Many scholarly studies blame congenital clubfoot on:
- Abnormal leg muscle development [rx]
- Connective tissue genetic defect [rx]
- The defective cartilaginous anlage of the anterior part of the talus [rx]
- Intra-uterine compression [rx]
- Dislocation of the talonavicular joint [rx]
- Defect in peroneal muscle innervation [rx]
- Defective anterior horn cells [rx]
- Abnormal tendon insertions [rx, rx]
- The arrest of development [rx]
- Tight deltoid ligament [rx] etc.
- Mutations in genes involved in muscle development are risk factors for clubfoot, specifically those encoding the muscle contractile complex (MYH3, TPM2, TNNT3, TNNI2 and MYH8). These can cause congenital contractures, including clubfoot, in distal arthrogryposis (DA) syndromes.[rx]
- Clubfoot can also be present in people with genetic conditions such as Loeys–Dietz syndrome. The PITX1-TBX4 transcriptional pathway has become key to the study of clubfoot. PITX1 and TBX4 are uniquely expressed in the hind limb.[rx]
Mechanical Factors in Utero
- This is the oldest theory and was first proposed by Hippocrates [rx, rx, rx]. He believed that the foot was held in a position of equinovarus by external uterine compression. However, Parker in 1824 and Browne in 1939 believed that diminution of amniotic fluid, as in oligohydramnios, prevents fetal movement and renders the fetus vulnerable to extrinsic pressure [rx].
Primary Germ Plasma Defect
Arrested Fetal Development
a) Intrauterine environment
b) Environmental influences
- The harmful influence of teratogenic agents on the fetal environment and development are well exemplified by the effect of rubella and thalidomide in pregnancy. Many authors believe that there are various environmental factors responsible for the appearance of a clubfoot, as there are various substances capable of producing a temporary growth arrest [rx, rx, rx].
The Mechanical Forces or Positional Hypothesis
- Promoted the widely held hypothesis of uterine restriction, believing that restriction of fetal foot movement by the uterus caused ICTEV. He suggested that ICTEV arose from oligohydramnios sequence, i.e. believing that reduced amniotic fluid volume is in itself a cause.
The Bone/Joint Hypothesis
- The bone/joint hypothesis postulates that positional bony abnormalities underlie the anomaly. Hippocrates wrote: ‘The deformity involves the entire combination of bones which make up the skeleton of the foot. All the changes seen in the soft part are secondary.
The Connective Tissue Hypothesis
- The connective tissue hypothesis suggests that a primary abnormality of the connective tissue is responsible for ICTEV. This is supported by the association of ICTEV with joint laxity [rx]. Affected children have marked plantar fibrosis at the surgery. Fetal studies give conflicting evidence. [rx] performed a systematic pathological study of 12 fetuses with ICTEV.
The Vascular Hypothesis
- They documented vascular abnormalities in ‘all deformed feet of 12 fetuses’. At the level of the sinus tarsi, there was the blocking of one or more branches of the vascular tree of the foot. This was ‘most conspicuous in the early period of fetal life, and reduced to a simple knot of fatty infiltration and fibrous tissue in older specimens and the stillborn’
Support for a Neurological Hypothesis
- Talipes equinovarus is a feature of many neurological syndromes; for example, it is often seen in association with neurological abnormalities that are secondary to spina bifida.
The Developmental Arrest Hypothesis
- During late normal human limb development (9–38 weeks), chondrification of the foot is completed, ossification commences, joint cavitation and ligament formation is completed and the distal limb rotates medially.
- Lifestyle choices – If you smoke or use illegal drugs while you’re pregnant, you raise your baby’s chances of being born with it.
- Other birth defects – In some cases, it’s linked with other another condition a baby is born with, such as spina bifida.
- Too little amniotic fluid during pregnancy – This surrounds your baby in the womb. If there’s not enough, your baby’s chances of being born with clubfoot are higher.
Symptoms of Clubfoot
- The top of the foot is usually twisted downward and inward, increasing the arch and turning the heel inward.
- The foot may be turned so severely that it actually looks as if it’s upside down.
- The affected leg or foot may be slightly shorter.
- The calf muscles in the affected leg are usually underdeveloped.
In an infant born with clubfoot
- The top of the foot twists downwards and inwards
- The arch is more pronounced and the heel turns inward
- In severe cases, the foot may look as if it is upside-down
- The calf muscles tend to be underdeveloped
- If only one foot is affected, it is usually slightly shorter than the other, especially at the heel
Diagnosis of Clubfoot
Diagnosis of clubfoot deformity is by physical examination. Typically, a newborn is examined shortly after delivery with a head to toe assessment. Examination of the lower extremity and foot reveals the deformity, which may affect one or both feet. Examination of the foot shows four components of deformity.
- First, there is a higher arch on the inside of the foot – This component of the deformity can occur without the other aspects of clubfoot deformity. In isolation, this aspect of the deformity is called cavus deformity.
- Second, the forefoot is curved inward or medially (toward the big toe) – This component of the deformity can occur without the other aspects of clubfoot deformity. In isolation, this aspect of the deformity is called metatarsus adductus.
- Third, the heel is turned inward – This is a natural motion of the heel and subtalar joint, typically referred to as inversion. In clubfoot deformity, the turning in (inversion) of the heel is fixed (not passively correctable) and considered a varus deformity.
- Fourth, and finally, the ankle is pointed downward – This is a natural motion of the ankle referred to as plantar flexion. In clubfoot deformity, this position is fixed (not correctable) and is referred to as equinus deformity.
In some cases, it may be possible to detect the disease prior to birth during a prenatal ultrasound. Prenatal diagnosis by ultrasound can allow parents the opportunity to get information about this condition and make plans for treatment after their baby is born.[rx]
Clubfoot sometimes diagnosed in utero
- 1st trimester – associated anomalies, including non-musculoskeletal ones, are very common in children diagnosed with clubfoot in the first trimester
- 2nd trimester – these are typically true clubfeet, but associated anomalies are less common
- 3rd trimester – if clubfoot first diagnosed in 3rd trimester, the false positive rate is higher due to a higher probability of intrauterine crowding
Treatment of Clubfoot
In general, the original correction may be recovered in four to six weeks with manipulations and plaster casts, changed every 14 days, holding the foot in marked abduction and as much dorsiflexion as possible at the ankle in the last cast. This treatment is followed by lengthening the tendon Achilles when dorsiflexion of the ankle is less than 15 degrees. A percutaneous tenotomy can be performed until one year of age. The last plaster cast is left on for three to four weeks. When the cast is removed, shoes attached in external rotation to a bar are worn at night and with naps, until the child is about four years old.
Treatment of recurrences
- Plaster casts
- Denis Browne splints
- Subcutaneous section, tendoachillis
- Tendo Achilles lengthening
- Anterior tibial transfer
- Recession, extensor hallucis longus
- Recession, extensor digitorum longus
- Subcutaneous plantar fasciotomy
- Medial release
- Lisfranc capsulotomy
Stretching and Casting
This is the most common treatment for clubfoot. Your doctor will
- Move your baby’s foot into a correct position and then place it in a cast to hold it there
- Reposition and recast your baby’s foot once a week for several months
- Perform a minor surgical procedure to lengthen the Achilles tendon (percutaneous Achilles tenotomy) toward the end of this process
After the shape of your baby’s foot is realigned, you’ll need to maintain it with one or more of the following
- Doing stretching exercises with your baby
- Putting your child in special shoes and braces
- Making sure your child wears the shoes and braces as long as needed — usually full time for three months, and then at night and during naps for up to three years
- Manipulation should be gentle but yet strong enough to stretch the soft tissue contractures. Forceful manipulation may result in a spurious correction producing rocker bottom foot.
- Traditionally as suggested by Hippocrates [rx], the components of clubfoot deformity were corrected from distal to proximal (i.e. correction of supination, forefoot adduction and followed by equinus).
- However, this concept is no longer popular, as equinus, varus and adduction deformities occur simultaneously and not as an isolated component. Thus, attempts are made to correct all elements of the deformities simultaneously.
- In Ponseti’s first case series he described the use of the foot abduction orthosis (FAO) after 3 months of full time bracing for an additional mean duration of 21 months (ranging from ten to 30 months) with a recurrence found in 56 % of cases [rx].
- Due to the high rate of recurrence in this first series he recommended the FAO to be used at night for at least five or six years in his second paper on club foot treatment [rx].
- Non-compliance has been recognized as a significant risk factor for the recurrence of club foot after correction with the Ponseti method with the parental educational level being an important factor [rx].
The Ponseti Technique
The corrective process utilizing the Ponseti technique can be divided into two phases
- The treatment phase, during which time the deformity is corrected, and
- The maintenance phase, during which time a brace is utilized to prevent a recurrence.
The treatment phase starts as soon as the skin condition of the child permits the use of plaster casts, till that time regular corrective manipulation of the foot by the mother is carried out. The treatment phase starts with the first cast aiming to align the forefoot with the midfoot and hindfoot. This is achieved by;
- Stabilizing the talus by placing the thumb over the lateral part of its head.
- Elevating the first ray to achieve supination of the forefoot in respect to the midfoot and hindfoot.
- Putting on a well-padded plaster cast by holding this position and molding it well.
In doing so, the cavus [rx] is corrected, typically after one cast.
- Stabilizing the talus by placing thumb over the lateral part of its head.
- Holding the supinated foot in abduction while applying the cast.
- Applying a well-padded plaster by holding the corrected position and molding it well.
- Another nonsurgical method to correct clubfoot incorporates stretching, mobilization, and taping. The French method — also called the functional or physical therapy method — is typically directed by a physical therapist who has specialized training and experience.
- Like the Ponseti method, the French method is begun soon after birth and requires family involvement. Each day, the baby’s foot must be stretched and manipulated, then taped to maintain the range of motion gained by the manipulation. After taping, a plastic splint is put on over the tape to maintain the improved range of motion.
- This method requires approximately three visits to the physical therapist each week. Because this is a daily regimen, the therapist will teach the parents how to do it correctly at home.
After 3 months, most babies have significant improvement in foot position, and visits to the physical therapist are required less often. Like children treated with the Ponseti method, babies treated with the French method commonly require an Achilles tenotomy to improve dorsiflexion of the ankle.
- With the Ponseti method, the vast majority of patients require Achilles tenotomy to correct residual equinus after casting. Multiple techniques have been described for tenotomy, each with their own risk profile.
- Some advocate for an open procedure performed in the operating room while others perform the procedure in recently, where Achilles tenotomy was performed in the operating room with a mini-open technique. All patients were discharged home the same day; none experienced adverse effects of anesthesia or complications from the procedure [rx].
New Techniques Without Tenotomy
- New techniques that are variations of the Ponseti method may one day provide an alternative to Achilles tenotomy for correction of equinus deformity. In one study, a dynamic dorsiflexion splint was applied to correct equinus after completion of serial manipulation and casting.
- Mean dorsiflexion improved nearly 15° at final follow-up with the use of a dynamic splint; however, 10 % of patients dropped out of the study group secondary to poor compliance and/or skin complications secondary to the splint [rx].
- Another non-surgical method of equinus correction involves the injection of Botox into the gastrocsoleus complex after serial manipulations and casting.
Tibialis Anterior Tendon Transfer
- Dynamic supination during gait and heel varus are common deformities that can occur after Ponseti casting and are usually attributed to poor brace compliance. Residual forefoot adductus after completion of casting [rx] and higher initial Pirani scores at the beginning of treatment [rx] increase the probability of recurrent deformity and subsequent surgical intervention.
- The tibialis anterior tendon transfer (TATT) has been described as an effective procedure to address recurrent deformity. Holt et al. demonstrated the long-term effectiveness of the TATT.
Posteromedial soft tissue release and tendon lengthening
- resistant and/or recurrent feet in young children which have failed Ponseti casting and bracing
- “rocker bottom” feet that develop following serial casting which failed non-surgical intervention
- syndrome-associated clubfoot if casting fails
- when performed, it is often done at 9-10 months of age in non-syndromic feet so walking is not delayed
- requires postoperative casting for optimal results
- long-term stiffness and pain are relatively common
- the extent of soft-tissue release correlates inversely with the long-term function of the foot and patient
Medial column lengthening or lateral column-shortening osteotomy, or cuboid decancellation
- often combined with initial surgical clubfoot release in children more than 2-3 years old
- may be performed in 3-10 years old children with recurrent deformity and “bean-shaped” foot
- in severe, rigid recurrent clubfoot in children with arthrogryposis
- age typically 6-10 years
Multiplanar supra malleolar osteotomy
- rarely necessary
- salvage procedure in older children with complex, rigid, multiplanar clubfoot deformities that have failed conventional operative management
- salvage procedure in older children (8-10 yrs) with an insensate foot.
Ring fixator (Taylor Spatial Frame) application and gradual correction
- complex deformity resistant to standard methods of treatment
- recurrence of deformity is very high after frame removal
- almost never indicated
- contraindicated in insensate feet due to rigidity and resultant ulceration
Clubfoot typically doesn’t cause any problems until your child starts to stand and walk. If the clubfoot is treated, your child will most likely walk fairly normally. He or she may have some difficulty with:
- Movement disorders – The affected foot may be slightly less flexible.
- Leg length – The affected leg may be slightly shorter, but generally does not cause significant problems with mobility.
- Shoe size – The affected foot may be up to 1 1/2 shoe sizes smaller than the unaffected foot.
- Calf size – The muscles of the calf on the affected side may always be smaller than those on the other side.
- Arthritis – Your child is likely to develop arthritis.
- Poor self-image – The unusual appearance of the foot may make your child’s body image a concern during the teen years.
- Inability to walk normally – The twist of the ankle may not allow your child to walk on the sole of the foot. To compensate, he or she may walk on the ball of the foot, the outside of the foot or even the top of the foot in severe cases.
- Problems stemming from walking adjustments – Walking adjustments may prevent natural growth of the calf muscles, cause large sores or calluses on the foot, and result in an awkward gait.