Introduction

Clubfoot is the most common deformity of the bones and joints in new-borns. It occurs in about 1 in 1,000 live births. The chances of having a second child with a clubfoot are approximately 1 in 30. The cause of club­foot is not exactly known, but it is most likely a genetic disorder with combination of unidentified environmental factors. Boys are affected three times more than girls. Clubfoot is bilateral in about half of the cases [1, 2].

Approach towards a clubfoot patient

Assessment (diagnosis, classification and grading): At the first consultation in the clubfoot clinic, perform a complete history, visual foot inspection, general and musculoskeletal physical examination.

Examine the cardiovascular, respiratory, gastro-intestinal, genito-urinary, and central nervous systems as well as the spine, upper extremities, hips, and feet to rule out other associated congenital abnormalities such as spina bifida (an open spinal cord), or arthrogryposis (multiple joint contractures) which suggest a syndrome. Then make a diagnosis of clubfoot, classify its type, and assess the severity of deformity by pirani score.

This information helps to guide treatment and reassure parents about the treatment plans [1,4,18].

Diagnosis of clubfoot: Congenital club foot has four components (CAVE) [1, 4, 18] (Figure 1):

Figure 1:Clinical photograph of CTEV child showing cavus (a), adductus (b), inversion (c) & equinus (d) (Drawn from Pirani S. Naddumba E. In: Staheli’s Ponseti club foot management. Teaching manual for healthcare providers. 2008;1:1-48)

Lateral Head of Talus (LHT): Uncovered talar head is estimated by abducting the deformed foot & palpating the talar head with thumb.

Normally, abduction reduces the navicular completely to talar head which is no longer palpable and scores 0. With medial contracture, navicular does not fully cover over the talar head & is partially covers but remains somewhat palpable& scores 0.5.

A talar head that does not cover at all & remains easily palpable scores 1.

Hind foot Score (HS): It measures hindfoot deformity and is sum of posterior crease, rigidity of equinus and emptiness of heel. Values can range from 0 (no deformity) to 3 (severe deformity).

Posterior Crease (PC): Normal posterior ankle skin shows multiple fine creases and scores 0. Posterior heel with one or two deeper creases scores 0.5. Single deep crease changing the contour of heel scores 1.

Rigid Equinus (RE): Normal ankle extends at least 15⁰ beyond neutral &scores 0. Ankle extension only to neutral scores 0.5. Extension short of neutral scores 1.

Empty Heel (EH): Normally calcaneus is palpable in extended ankle at heel and scores 0. A calcaneus that is palpable only deeply scores 0.5 and a calcaneus that is not palpable scores 1.

Total Score (TS): This is the sum of the HS and MS and indicates the amount of deformity overall. Values can range from 0 (no deformity) to 6 (severe deformity).

Pirani Score Assessment [4,20]

Typical clubfoot: initially has HS of 3 and MS of 3 (TS of 6), which at 2 to 4 weeks shows decreased MS but same HSe. at this time midfoot pathology is corrected(MS< 1) but hindfoot pathology persists (HS> 1).

At this stage, Achilles tendon tenotomy is performed to correct hindfoot pathology and after tenotomy HS improved to 0.5, MS remained 0.5, & TS improved to 1(figure 2).

History of Clubfoot Management

Treatment has been variable and controversial depending on the era [2,4].

Early treatment: First treatment was given by Hippocrates, who recognized the importance of starting the treatment early, before the bony deformity occurs [1,4]. William Cheselden at St. Thomas’s Hospital, London was first to apply cast made of egg-white-soaked bandages.

In 1820’s Delpech, Stomeyer and Little were first to do Achilles tenotomy. Plaster of Paris casts were first used by M. Guerin in 1833. William Adams was pioneer to describe clubfoot pathology after dissecting stillborns [1,4]. In 1930, Kite advocated good correction by sequentially correcting the clubfoot deformity, but it took prolonged correction time (average 22 months) as it failed to take advantage of synchronous movements of tarsal bones at subtalar joint [5].

Surgical methods: Advent of antisepsis, anaesthesia and surgical tourniquet permitted surgical correction of deformity. In 1890, Extensive postero-medial releases were developed by Phelps, Duval, Ogston, and others. But in 1950, Steindler showed poor results (painful & stiff foot) in more than 50% of surgical cases, leading to a reappraisal of nonsurgical methods [4,6,7].

Ponseti method: With advent of Ponseti method in 1990s, without doubt, the primary treatment of CTEV has remarkably shifted to Ponseti method all over the world [8-11].

Rational of Ponseti method

Ponseti’s treatment is based on sound understanding of the functional anatomy of foot and biological response of young connective tissue and bone to change in direction of mechanical stimuli which can simultaneously correct cavus, adductus, and varus followed by correction of equinus, in mean duration of 5 weeks. He took advantage of synchronous movements of the tarsal bones at subtalar joint to unlock the deformity [4,12,13].

Patho-anatomy, biology and kinematic coupling by Ponseti

In 1966, with dissection of stillborn club feet, Ponseti revealed abnormalities in tarsal bones, tendons and ligaments, posteriorly and medially and abnormal intertarsal relationships, particularly navicular (Figure 7). Muscles of the foot and leg were smaller, thick and taut than normal. According to him CTEV foot, fails to pronate, because the calcaneum cannot abduct, evert and dorsiflex, as it is locked under the talus in adducted, flexed and supinated position [13,14].

Acceptance of Ponseti Method

Ponseti performed clinical trials of his technique of manipulation and casting on several occasions (1963, 1972, 1980, 1992), but since the pathology and biology was poorly understood by clinicians, they did not adopt his method. Further his reports were not given due credit because they were not read accurately.

It was only in 21st century that his technique was widely accepted, because of very satisfactory long-term outcomes by independent observers, documented clinical proven effectiveness, MRI proven studies, parent demand of non-surgical treatment and re-emphasis on technical aspects along with clarification of common errors. It is universally accepted now that best treatment for clubfeet regardless of cost is Ponseti Method [8-11].

Ponseti manipulation and casting [1,4,18,21,22]

The foot is most receptive at or within a few weeks after the birth for Ponseti treatment by straightening the clubfoot by gently pushing the foot towards the correct position and retaining the corrected position applying a holding cast. Hence treatment should be started earliest.

Cast (average 5 to 7 casts) are changed weekly or biweekly with additional corrections added at each visit. Correction is completed by a small cut just above the heel before the final cast, followed by bracing until 4 years of age to prevent relapse.

1. Attitude:Be kind, sensitive and reassure the parents making them understand overview of management and the importance of each step.

2. Position:Casting requires two skilled persons – a manipulator and an assistant along with the mother to hold the infant in her lap. All should be seated, with manipulator sitting laterally and assistant medially, with respect to affected foot, both facing the foot.

3. Manipulation and casting:The manipulator manipulates the foot to correct the deformity as much as possible without hurting the infant to stretch medial and plantar soft tissues.

To do this, the manipulator’s left hand moves up the leg and correction is maintained by gentle left thumb pressure over the upper leg.

The manipulator’s right hand continues holding the forefoot in the corrected position by applying some pressure to the talar head laterally and the assistant applies the padding and the cast over the foot and leg beginning from toes to knee (Figure 8). If the child is at discomfort, then slow down, reduce pressure and allow child to calm.

Correct equines (5th cast): after achieving 70⁰ abduction start correcting equinus by dorsiflexion of foot or it may need percutaneous tenotomy of the tendoachillies. Full abduction correction can usually be achieved with cast 5.

1. Moulding:The manipulator stabilizes the knee and holds foot in corrected position while the assistant moulds the cast by his left hand at the arches and bony prominences and right hand give counterpressure. The assistant’s hands and fingers move continuously to reduce the risk of pressure sores while the plaster sets. Moulding causes the cast to fit snugly, gives appearance of the foot and prevents slippage, pressure sore or secondary deformity.

2. Above knee extension: Once lower section is set, the cast is extended above the flexed knee (90 degrees) to avoid the possibility of the cast slipping off the leg.

3. Trimming:The child is cleaned and cast is trimmed to remove excess plaster to leave the toes uncrowded but supported [H].

4. Instruction: Parents are reassured, encouraged and asked to regularly observe toes for change colour/swell, swelling, baby cries, or not feeding or the cast gets wet. In case, this happens instruct them to revisit or soak the cast and remove it immediately.

5. Cast removal: Casts should be removed in clinics just before a new cast is applied else correction can be lost from the time the cast is removed until the new one is placed.

Initially soak the cast in water for about 20 min, then softened cast can be removed by unrolling the plaster (easiest if end knob is found), by plaster shear blade, plaster knife or scalpel cutting obliquely. Electric cast saw are not recommended. Start cutting from anterior or lateral side of groin moving downward cutting only cast not the padding, removing first above knee part followed by below knee part (Figure 9).

6. Cast appearance:With each cast foot shows improvement and progressively abducts 20⁰ each time. After 4th cast, full cavus, adductus, and varus correction is seen. Equinus also gradually improves because the calcaneus dorsiflexes as it abducts under the talus sono direct equinus correction is required, but it may require a percutaneous tenotomy of the achilles tendon. Attempted dorsiflexion against tight tendo-achilles causes a rocker bottom foot deformity.

7. Complications of casting:superficial and deep pressure sores can occur specially at talar head or heel. Superficial sores are managed by applying a dressing and a new cast with additional padding. Deep sores are dressed and left out of the cast for one week to allow healing and after healing Casting is resumed.

usually about one to one and a half centimetres above the calcaneus, and infiltrating a small amount of local anaesthetic just medial to this point. Be aware that too much local anaesthetic makes palpation of the tendon difficult and the procedure more complicated.

3. Technique of Heel cord tenotomy:Insert the tip of the scalpel blade from the medial side, directed immediately anterior to the tendon. Keep the flat part of the blade parallel to the tendon. The initial entry causes a small longitudinal incision.

Care must be taken to be gentle so as not to accidentally make a large skin incision. The blade is then rotated, so that its sharp edge is directed posteriorly towards the tendon. The blade is then moved a little posteriorly and a “pop” is felt as the sharp edge releases the tendon.

The tendon is not cut completely unless a “pop” is appreciated. An additional 15⁰ to 20⁰ of dorsiflexion is typically gained after the tenotomy. A small amount of bleeding is normal. A piece of clean gauze is placed over the incision and above-knee plaster cast applied.

4. Post tenotomy cast:After correction of equinus by tenotomy, with the foot abducted 60⁰ to 70⁰& 15⁰ dorsiflexion, last cast is applied for 3 weeks. The foot looks overcorrected with respect to the thigh. It should be replaced if it softens or becomes soiled before 3 weeks. Usually no analgesic is necessary.

5. Cast removal:After 3 weeks, the cast is removed and20⁰ of dorsiflexion is now possible and foot is ready for bracing.

Figure 10:Clinical photographs showing steps of tenotomy (a to d)(Drawn from Pirani S. Naddumba E. In: Staheli’s Ponseti club foot management. Teaching manual for healthcare providers. 2008;1:1-48)

Step 4: Bracing

Bracing is in-dispensable for preventing relapse of deformity by holding the foot in the corrected position. Follow-up at regular intervals for compliance of brace is essential and critical for successful management. Brace should hold the foot abducted and dorsiflexed – hence the term foot abduction brace (Figure 11)[4,18,21,22].

7. Time and duration: It should be used full time for 3 months and at night time until 4 years of age regularly and without skipping even one day.

8. Availability: Stock FABs in the clubfoot clinic so that they are available for immediate initial fitting and replacement, reducing relapse and transportation costs.

9. Follow-ups: should be done at regular intervals to minimize problems and to detect relapse early. First at 2 weeks and then every 3 monthly till 2 years’ age and then 6 monthly till 4 years’ age.

10. Discontinue: brace can be discontinued after age of 4 years if there is no sign of relapse. If there is any sign of relapse, reapply a series of corrective casts.

11. Compliance:Failure to comply with bracing protocols is the most common cause of relapse. To prevent relapse, educate, demonstrate, reassure and ensure consistency.

Outcome of Ponseti Method
Correctly applied cast is successful in almost all the cases, and it depends on experience of treating team, compliance of family and degree of stiffness of foot. Feet treated by this method are strong, flexible, and pain free, allowing a normal life, although affected feet and leg may be slightly shorter and narrower than the normal foot, but it makes no difference [8-11, 19].

Recommended visits
About total 20 visits over four years leads to optimal outcomes.

Corrective phase:requires 7 visits – 5 weekly visits for five casts, 6th visit after 3 weeks of tenotomy for fitting of FAB, and 7th visit 2 weeks later for brace-wearing compliance check.

Maintenance phase: every 3 monthly till walking age, and then every 6 months till 4 years’ age for assessment of relapse and brace compliance.

Development & modification in Ponseti technique
The technique is equally effective in hands of paramedical hands and corrects deformity even in older child, complex, atypical and syndromic clubfeet. But it is complicated and may take longer duration (average 10 to 12 casts).Accelerated protocol with cast changes every 4 to 5 days can shorten duration time [23].

Errors or cause of failure in management [24]

1. Failure to recognize clubfoot: is a result of lack of awareness of clinical features of clubfoot or knowledge that it can be treated. Can be overcome by education, awareness and performing visual foot inspection.

11. Failure to recognize relapse early: Relapse can be identified early by observing the child’s gait, which shows early heel rise (child walking away from examiner), swing phase dynamic supination (child walking towards examiner) and loss of ankle dorsiflexion to less than 10⁰. These relapsed can be managed by repeat corrective casting.

12. Management without bracing: Avoiding the brace entirely is tempting and has shown 90% relapse rates at age of 1 year. Relapse is rare after 4 years, hence bracing until 4 years of age is necessary to reduce relapse.

13. Attempts to obtain perfect anatomical correction: It is impossible to achieve complete anatomic correction by Ponseti treatment. Long-term follow-up radiographs show some abnormalities, but it does not correlate with long term, good clinical and functional, supple and plantigrade foot, obtained by Ponseti method.

14. Non-compliance:irregular follow up in casting phase, not using brace until 4 years, will cause relapse.

Other Clubfeet Situation

Most idiopathic congenital clubfeet correct with about five well-applied Ponseti casts. Some clubfeet, however, can be considered “difficult” as they have some unique characteristics that demand a modified approach for management [1,4,18,21,22,25].

Relapse [1,4,18,21,22,25]

Ponseti method corrects deformity, but it does not remove the cause and so it has tendency to recur.

1. Cause:It is almost always due to failure or incorrect bracing and may indicate neuromuscular disorder.

2. Age of presentation:relapse occurs before 5 years of age and is very rare after age 7.

3. Sequence:Early relapse presents as a loss of dorsiflexion, later, heel varus and adductus develop and rarely significant cavus may recur. Early relapse is therefore easier to correct than late relapse.

4. History: take history relating to bracing difficulties (pain, inconsolable crying, sore areas,) that led to inconsistent use or improper use like heel not touching down on the footplate of the shoe.

5. Signs of relapse:are different for before walking and after walking age.

6. Before Walking Age: Suspect relapse if the brace has incorrect angle of abduction and dorsiflexion, if the talar head remains palpable with the foot in maximal abduction,

Transfer the tendon to the dorsolateral incision under the extensor retinaculum and extensor tendons. Localize lateral cuneiform radiographically and make a drill hole in the middle of the lateral cuneiform large enough to accommodate the tendon. Thread a straight needle on each of the securing sutures and pass both needles into the hole and penetrates the sole of the foot, with needles Passing through different holes of secure button placed plantarly, with foot held in dorsiflexion, pull the tendon into the drill hole by traction on the fixation sutures and tie the fixation suture with multiple knots.

6. Supplemental fixation: Supplement the button fixation by suturing the tendon to the periosteum at the site where the tendon enters the cuneiform, using a heavy absorbable suture.

7. Local anaesthetic and closure: Inject long-acting local anaesthetic into the wound to reduce immediate postoperative pain and close wound with absorbable subcutaneous sutures.

8. Cast immobilization: apply a long-leg cast with foot in abduction & dorsiflexion.

9. Postoperative care: Usually, the patient remains hospitalized overnight. Remove the cast and button at 6 weeks. The child may mobilize weight-bearing as tolerated.

10. Bracing and follow-up: No bracing is necessary after the procedure. See the child again in 6 months to assess the effect of the transfer.

Figure 12:Clinical photograph of tibialis anterior transfer for dynamic supination showing incision (a), tibial anterior harvest (b), transfer of tendon to cuneiform (c) and securing tendon plantarwards (d). (Drawn from Pirani S. Naddumba E. In: Staheli’s Ponseti club foot management. Teaching manual for healthcare providers. 2008;1:1-48)

Following this start abducting foot with counter press at lateral talar head. Apply casts with the above-knee portion in 110⁰ flexion to prevent slippage. Average10 casts can be needed.

5. Tenotomy: A tenotomy is necessary in all cases. Perform the tenotomy when plantaris corrected. At least 10⁰ dorsiflexion is necessary. Sometimes it is necessary to change casts at weekly intervals after the tenotomy to gain more dorsiflexion, if sufficient dorsiflexion is not achieved immediately after the tenotomy.

6. Bracing: Reduce abduction on the affected side to 40⁰ in the foot abduction brace. The follow-up protocol remains the same.

Syndromicclubfoot

Congenital abnormalities, such as arthrogryposis, myelomeningocoele, and other syndromes, often have abnormal collagen forming their ligaments, capsules, and other soft tissues.

Hence syndromic clubfeet are difficult to treat. Many syndromic clubfeet, correct and plantigrade foot can be achieved with standard Ponseti method however; correction may require more casts than usual. Long-term functional outcome usually depends more on the underlying syndrome than the clubfoot [1,4,25].

Resistant clubfoot

Rarely, idiopathic congenital clubfeet do not correct completely with accurately applied Ponseti treatment due to co-existing pathology such as tarsal coalition the surgical correction by posterior or posteromedial release is necessary [1,4,25].

Conclusion

Neglected club foot can affect adversely on functions and cosmetic appearance; hence treatment should start at earliest as possible. Ponseti method, based on sound understanding of functional anatomy and biological response of tissue ,showed excellent results with nonsurgical serial manipulation and casting, simultaneously correcting all the deformities.

Cavus is corrected in first cast by supinating the foot followed by correction of varus and adduction, by abduction the foot, with counter pressing lateral talar head, followed by correction of equinus by percutaneous tenotomy in mean 5 weeks.

Post treatment bracing is necessary to prevent the relapse till age of 4 years. The technique is equally effective in hands of paramedics, in older child, complex atypical and syndromic clubfeet, although it may take longer duration to correct. Feet treated by this method are strong, flexible, and pain free, although affected feet are slightly shorter and narrower.