CTEV (only idiopathic club foot) all below 2 years of age treated conservatively with Ponseti technique

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1 International Journal of advances in health sciences (IJHS) ISSN Vol1, Issue3, 2014, pp A case report CTEV (only idiopathic club foot) all below 2 years of age treated conservatively with Ponseti technique Vishal Moudgil Punjab Institute of Medical Sciences Jalandhar, India [Received-07/10/2014, Accepted-15/10/2014] ABSTRACT Among the vast treasures of King Tut, the archeologists found wall murals depicting a rather grotesque dwarf. Clubfoot or Congenital Talipes Equinovarus is one of the most common congenital and perhaps the oldest documented deformities. It is a rigid deformity having three components, namely: Ankle and foot equinus, Hindfoot varus and Forefoot abduction. About 1 in every 1000 children is born with clubfoot worldwide. A normal developing foot turns into a clubfoot around the 3rd month of foetal life. Etiological factors for the development of the deformity seem to be active for several years explaining relapses after correction.though the management of CTEV with manipulative casts dates back to the day of human evolution its been neglected for centuries. Developing countries like India with an agrarian culture requires walking on uneven surfaces, which require supple and painless foot. Also with limited financial resources operative management become difficult. Ponseti technique is simple, effective cheap and non invasive.clubfoot children are often abandoned or face a future in poverty. By correcting their feet, they get the opportunity to live a normal life.this controversy in the management of CTEV has stimulated me to study this subject and its management and discuss its results using manipulative casts by Ponseti technique. Materials and methods This is a prospective study of 75 patients with CTEV (only idiopathic club foot) all below 2 years of age treated conservatively with Ponseti technique and evaluated with the help of Columbian Clubfoot Score according to a standard proforma. All the patients were treated in out patient department and were given 5-6 manipulative casts at a weekly interval after which Tendo Achilles and a full corrective cast was given for 3 weeks after which they were put on Steenbeek s brace. In our series, we have 75 patients (101feet), in 49 there was unilateral affection and 26 had bilateral affection for study, which were treated with PONSETI CAST and tenotomy and correction maintained in Steenbeek s brace. Conclusion C.T.E.V. is a congenital development disease, which if treated early by conservative treatment has excellent results.males predominance noted with a ratio of 4:1.There was seen an increase in incidence coming 1 relatives with 11% having positive family history.the Ponseti method is a very effective and relatively inexpensive treatment with good long term results. INTRODUCTION The earliest documentation of Congenital Talipes Equinovarus, better known as the Clubfoot comes from the egyptians who depicted the deformity in their wall murals, its treatment, however, was first

2 described in India as early as 1000 BC. Hippocrates gave us the first written documentation of clubfoot (circa 400 BC), implicating mechanical pressure as the cause. He was perhaps the first advocate of the manipulative correction of the deformity and was popularised the practice of initiating the treatment as soon after birth as possible. His technique involved serial manipulation of the deformed foot followed by application of strong bandages, which is eerily similar to the methods being employed now. He even laid emphasis on overcorrection to prevent recurrence. Two grading systems are commonly used for clubfoot the Pirani score and the Dimeglio grade. The Pirani score is outlined in box 2. A correlation has been shown between the Pirani score and subsequent need for Achilles tenotomy.[1] Clubfoot is increasingly diagnosed on prenatal scans and these have a positive predictive value of around 85%.[2] The outcome of surgery is available, and most evidence comes from case series. One case series published in 2006 looked at 73 feet in 45 patients with a minimum follow-up of 25 years. This was a thorough review with the patients completing three independent quality of life questionnaires, including the Laaveg and Ponseti functional score, which is commonly used in clubfoot outcome studies.[3 ]On this measure, 34 feet (47%) had a poor outcome and most had more than one operation.[4] In a case series looking at staged surgical release, 99 feet in 71 patients with severe clubfoot were studied. The average follow-up was 11.5 years and the relapse rate was 76%.[5] Gait analysis has been used by some investigators to try to quantify the results of treatment. A case-control study using this technique compared the outcomes of surgical and non-surgical treatment. This showed that surgery led to a greater proportion of gait abnormalities.[6] These teachings and practices were quickly forgotten in the Middle-Ages, where the management of CTEV was the province of barbersurgeons, charlatans and bonesetters. The next documented treatment was repeated stretching devised by Arceus who also invented two mechanical devices for maintaining the correction. Cheselden also advocated repeated stretching in the 18th century while Scarpa in 1803 attempted to treat the deformity by forceful manipulation and subsequent wearing of what was then known as Scarpa s shoe, a device similar to the one developed by Arceus. Timothy Sheldrake in 1806 described in his essay Distortions of Leg and Feet of Children, a technique similar to the one used by Hippocrates. He was of the view that even after correction of the deformity the foot should not be left free for the fear of recurrence. Delpech, in 1823 performed subcutaneous tenotomy of Achilles tendon, sepsis ensued in both the patients and he never repeated the procedure. Stromeyer however continued the procedure, subsequently performing it on W.J. Little, a British Surgeon who acquired equinovarus secondarily to Poliomyelitis. Stromeyer taught Little the procedure, which Little later introduced to England with resounding success. W.J. Little was vehemently against the mechanical theory of CTEV, and was of the view that it occurred due to abnormal contractions in the IU life.this view point lead to use of irons extending from foot to the hip for the treatment of CTEV. Rogers and Dickson brought the technique to USA. Adams in the second half of the 19th century performed dissections on stillborns, documenting the fact that the only bone which exhibited marked variation from the normal was Talus. He drew the conclusion that CTEV was anatomically dislocation of talocalcaneonavicular joint, and recommended early surgery to achieve anatomical reduction. M. Guerin was the first to use Plaster of paris to correct CTEV. In 1891, Phelps not only divided the Achilles tendon, but carried out a medial release of all soft tissues, elongation of the tibialis posterior and division of the medial ligament of the ankle joint Vishal Moudgil 85

3 and plantar fascia, abductor hallucis, flexor hallucis longus, all the short flexors and finally performed osteotomy of the neck of the talus and wedge resection of the calcaneus. Duval (1890), Ogston (1902) and Lane (1893) all carried out similar radical procedures. Elmslie and Ober believed only talonavicular capsule, the plantar fascia, the Achilles and posterior tibial tendon were the cause of CTEV while Brockman divided the abductor hallucis, tibialis posterior and subsequently carried out elongation of the Achilles tendon to correct the equinus. It is estimated that more than 100,000 babies are born worldwide each year with congenital clubfoot. Eighty percent of the cases occur in developing nations. Most are untreated or poorly treated. Neglected clubfoot causes crushing physical, social, psychological, and financial burdens on the patients, their families, and the society. Globally, neglected clubfoot is the most serious cause of physical disability among congenital musculoskeletal defects. Its one of the most common congenital deformities and associated with neuromuscular diseases, chromosomal abnormalities, Mendelian and non- Mendelian and extrinsic causes. The etiology and treatment is still controversial with no universal standards and with no standardized methods of evaluating results. There is no universally acceptable surgical procedure to correct it and no agreement regarding optimum age for surgery. There are strong advocates of both conservative and surgical treatment. This is all compounded by the fact that although there are numerous welldescribed synonyms which differentiate mild and severe virgin clubfoot there is neither consensus nor reliability in current methods of clubfoot assessment.in the middle of the 17th century, Arcaeus, Pare, and Fabrig recommended repeated stretching by the use of mechanical corrective apparatuses, which gradually eliminated the deformity with a turn-buckle. In 1963, in the Journal of Bone and Joint Surgery, the first article by Dr. Ignacio Ponseti was published but was disregarded. An article on congenital metatarsus adductus, published in the same journal in June 1966, was easily understood, perhaps because the deformity occurs in one plane but clubfoot was a triplanar deformity. In 1965 Dr. Ignacio Ponseti and Dr. Hiram Kite gave a lecture in Caracas, Venezuela, during which they applied plaster casts but with different techniques. After this during the long period of 1970 till 1995 many orthopaedics surgeons studied and began using Ponseti technique and only after the publication by Cooper DM, Dietz FR Treatment of Idiopathic Clubfoot: A Thirty-year follow-up Note in the Journal of Bone and Joint Surgery 77(10) : in 1995 and the publication of Congenital Clubfoot: Fundamentals of Treatment. Ponseti IV in 1996 was this technique being more and more accepted. Along with it in most of the developed countries many children with clubfoot underwent corrective surgery with disturbing, failure and complication. The need for one or more revision surgeries was common. Although the foot looked better after surgery it was stiff, weak, and painful. After adolescence, pain increased and often became crippling. A study of histological sections of ligaments from virgin clubfeet, obtained in the operating room and from fetuses and stillborns, revealed that the abundant young collagen was wavy, was very cellular and could be easily stretched. This was supported by thehuson thesis. It is now recognized that one of the major endeavours for the next few years must be establish a common set of criteria for evaluation of un operated CTEV Though the management of CTEV with manipulative casts dates back to the day of human evolution its been neglected for centuries. Developing countries like India with an agrian Vishal Moudgil 86

4 culture requires walking on in even surfaces, which require supple and painless foot. Also with limited financial resources operative management become difficult. Ponseti technique is simple, effective cheap and non invasive. This controversy in the management of CTEV has stimulated me to study this subject and its management and discuss its results using manipulative casts by Ponsetti technique. MATERIALS AND METHODS We have treated 175 patients with clubfoot with different aetiology including idiopathic clubfoot, teratologic clubfoot, arthrogryposis multi plexa, mengiomyocoele, neglected clubfoot. This is a prospective study of 75 patients with CTEV (only idiopathic club foot) all below 2 years of age treated conservatively with Ponseti technique and evaluated with the help of Columbian Clubfoot Score according to a standard proforma. All the patients were treated in out patient department and were given 5-6 manipulative casts at a weekly interval after which Tendo Achilles and a full corrective cast was given for 3 weeks after which they were put on Steenbeek s brace. This brace was worn continuously for 24 hours till the patient started walking. After the patient started walking he was put on pre walker CTEV shoes and later on Tarso Pronator shoes till skeletal maturity. Every patient was follwed up every monthly till 6 month and there after every 3 months for an average of 2 years. i) General Principles The method for assessing the amount of deformity in the congenital clubfoot deformity has been done using 6 well-described simple clinical signs. In validity of this scoring system was evaluated by correlating the MR findings and clinical scores (Pearson correlation) and all correlation were highly significant confirming the validity of this scoring system. In correlation with using this scoring during treatment it charts whether deformity is correcting and indicates area of residual contracture. The literature quotes a number of clinical signs. Six of them were chosen, 3 of these were chosen as they were thought to indicate area of residual contracture. These signs are those that change in severity as foot deformity change. Scoring of each of these signs has been done as : 0 = No abnormality 0.5 = Moderate Abnormality 1 = Severe Abnormality These six signs are divided by area of involvement into 2 groups of 3 signs: 1. Hindfoot contracture (HFC) Posterior Crease (PC) Empty Heel (EH) Rigid Equinus (RE) 2. Midfoot contracture (MFC) Curvature of Lateral Border of Foot (CLB) Medial Crease (MC) Lateral part of the Head of the Talus (LHT) Hence, each foot can receive : A Hindfoot contracture score(hfcs) between 0 and 3 A Midfoot contracture score(mfcs) nbetween 0 and 3 A Total score(ts) between 0 and 6 A higher score indicates a more severe deformity. Scoring the foot at each visit during treatment easily enables the treating orthopaedic officer or physiotherapist to document how the foot is responding to manipulation and casting. TECHNIQUE OF EXAMINATION: The examiner is seated. The infant is on mother s lap. A feeding relaxed infant allows for a more precise examination. The Signs: i) Curvature of the Lateral Border of the foot (CLB) The lateral border of the foot is normally straight. A curved border implies medial contracture. The amount of curvature indicates the amount of medial contracture. Vishal Moudgil 87

5 ii) Medial Crease of the foot (MC) Normally the skin of the area of the foot displays multiple fine lines. Deeper skin creases may imply more medial contracture. Look at the plantar surface of the foot and gauge the curvature of the lateral border. Normally the lateral border of the foot is straight from the heel to the head of the 5 th metatarsal. A straight edge placed along the lateral border of the hindfoot may assist in the estimation of the curvature. A straight lateral border of the foot is given a score of 0. Look at the medial arch of the foot with the foot in the position of maximum correction. Normally one sees multiple fine creases that do not change the contour of the arch and the MC sign is scored 0. In the abnormal foot the lateral border of the forefoot moves away from the straight edge. A mildly curved lateral border is scored 0.5 (the curvature appears to be in the distal part of the foot in the area of the metatarsals) In the abnormal foot there are one or two deep creases. If these deep creases do not appreciably change the contour of the arch, the Medial Crease sign is scored as a 0.5. A pronounced curvature of the lateral border is given a score of 1. (The curvature appears to be at the level of the calcaneocuboid joint.) If these deep creases appreciably change the contour of the arch, the MC sign is scored as a 1. Vishal Moudgil 88

6 iii) Posterior Crease of the ankle (PC) Normally the skin of the posterior ankle shows multiple fine creases - Deeper creases imply more posterior contracture. If these deep creases appreciably change the contour of the heel, the Posterior Crease sign is scored as 1. iv) Lateral part of the Head of the Talus (LHT) The talar head is uncovered laterally in the untreated clubfoot. As the deformity corrects the navicular reduces onto the head of the talus and covers it. This sign is a measurement of how far the navicular reduces onto the talar head. Look at the back of the heel. Normally one sees multiple fine creases that do not change the contour of the heel. They allow the skin to adjust and stretch as the ankle dorsiflexes. In this case, the Posterior Crease sign is scored as 0. In the abnormal foot there are one or two deep creases. If these deep creases do not appreciably change the contour of the heel, the Posterior Crease sign is scored as 0.5. Hold the foot deformed and palpate the lateral part of the head of the talus, with the pulp of the thumb. It lies anterior to the tip of the fibula. Abduct the foot with the other hand and note if the navicular reduces onto the head of the talus. The LHT sign is scored 0 if there is complete reduction of the navicular onto the head of the talus as indicated by loss of the ability to palpate the lateral edge of the head of the talus in the sinus tarsi. The LHT sign is scored 0.5 if there is partial reduction of the navicular onto the head of the talus as indicated by reduction of the ability to palpate the lateral edge of the head of the talus in the sinus tarsi. The LHT sign is scored 1 if there is fixed medial subluxation of the navicular as characterised by an easily palpable talar head, even with the forefoot in as much correction as is allowed by the deformity. v) Emptiness of the Heel (EH) This sign is a measure of posterior contracture. With the talus fully plantarflexed, the calcaneus is also in equinus and the posterior aspect of the calcaneus is drawn up and out of the heel pad. As Vishal Moudgil 89

7 the talar plantar flexion corrects, the calcaneus fills the heel pad. As the talar plantar flexion corrects, the calcaneus fills the heel pad. Palpating the heel pad therefore can give an estimation of how plantar flexed the talus is, and hence, an estimation of residual posterior contracture. The RE sign is scored 0 when the ankle dorsiflexes fully (the dorsum of the foot almost touches the front of the shin.) The examining finger is placed on the corner of the heel. Gentle pressure is applied with the finger. Normally the tuberosity of the calcaneus is immediately palpable and the empty heel sign is scored 0. The RE sign is scored 0.5 when the ankle dorsiflexion is not full but sufficient to allow the lateral border of the foot to make an angle of 90 degrees or less with respect to the long axis of the leg as viewed from the lateral side. The empty heel sign is scored 0.5 when the heel pad feels somewhat empty to the examining digit. The tuberosity however, remains palpable deep within the heel pad. The empty heel sign is scored 1 when the heel pad feels empty to the examining digit. No bony prominences can be appreciated within the heel pad. vi) Rigidity of Equinus (RE) This sign is a measure of the posterior contracture. The more the residual posterior contracture, the less the foot will dorsiflex. The RE sign is scored 1 when the ankle dorsiflexion is severely limited and the lateral Vishal Moudgil 90

8 border of the foot makes an angle of greater than 90 degrees with respect to the long axis of the leg as viewed from the lateral side. C) Recording the Information This is done on a chart and the scores are seen every follow up of the patient and recorded on a graph for comparison of score with the last visit. As the treatment continues the score keeps dropping. The chart and the graph are attached in the proforma. Discussion Classification of CTEV 1. Congenital or Idiopathic 2. Teratologic 3. Postural Etiopathogenesis of Idiopathic Clubfoot Primary Germplasm and Irani and Sherman theory defect occurs within fertilized germ cell as theorized by Irani and Sherman, which affects head of talus. Osteogenic theory or Arrested fetal development A. Victoria Diaz/is Fetal Position Embryonic Position Heel Varus At One Year Tibial Phase Fibular Phase Victoria Diaz Theory Fibular and tibial phases of rapid growth with gradual decrease in normal Theory of arrested development at about 7th- 8th week of gestation. If it occurs at 7th 8thweek marked defect after 9th week moderate. This theory of arrest is related to change in genetic factor called CRONON. The cronon guides precise timing of progressive modifications of every structure that undergoes during its development. Thus clubfoot is caused by disruptive elements, which are local or general that change the cronon due to intrauterine crowding. Environmental influences Occurring during 7th 8th week of pregnancy or exposure to some teratogenic agents and use of contraceptive pills is also associated with increased rate of incidences. Heredity Studies by Palmers, Wynnedavis have shown increased incidences of clubfoot among relatives of and most investigators believe that congenital idiopathic clubfoot is inherited as polygenic multi factorial trait. Heredity and Environment combined Wynne Davis states that inheritance is more susceptible to influence of environment factors thereby supporting the theory of hereditary predisposition and environment. The further Vishal Moudgil 91

9 support of this combination theory is provided by Idelbeerger study. The combined hypothesis maintains that some intrauterine factor in conjunction with hereditary predisposition causes a disturbance in development at a critical stage of embryonic development of foot thereby causing an arrest of normal fetal development. ETIOLOGY OF TERATOLOGIC CLUBFOOT The clubfoot associated with tetratologic disorder or syndrome 1. Arthrogryposis multiplexa congenita 2. Cerebral palsy 3. Chromosomal abnormalities 4. Diastrophic dwarfism 5. Freeman Sheldens syndrome 6. Muscular dystrophy 7. Myelodysplasia 8. Purine Robin Syndrome 9. Spinal muscular Etrophy DEFINITIONS AND PATHOANATOMY The treatment of clubfoot is based on the biology of the deformity and of the functional anatomy the foot. Biology : Clubfoot is not an embryonic malformation. A normally developing foot turns into a clubfoot during the second trimester of pregnancy. Clubfoot is rarely detected with ultrasonoraphy before the 16th week of gestation. Therefore, like developmental hip dysplasia and idiopathic scoliosis, clubfoot is a developmental deformation. The shape of the tarsal joints is altered relative to the altered positions of the tarsal bones. The forefoot is in some pronation, causing the plantar arch to be more concave (cavus). Increasing flexion of the metatarsal bones is present in a lateromedial direction. In the clubfoot, there appears to be excessive pull of the tibialis posterior abetted by the gastrosoleus, the tibialis anterior, and the long toe flexors. These muscles are smaller in size and shorter than in the normal foot. In the distal end of the gastrosoleus, there is an increase of connective tissue rich in collagen, which tends to spread into the tendo Achillis and the deep fascia. In the clubfoot, the ligaments of the posterior and medial aspect of the ankle and tarsal joints are very thick and taut, thereby severely restraining the foot in equinus and the navicular and calcaneus in adduction and inversion. The size of the leg muscles correlates inversely with the severity of the deformity. In the most severe clubfeet, the gastrosoleus is seen as a muscle of small size in the upper third of the calf. Excessive collagen synthesis in the ligaments, tendons, and muscles may persist until the child is 3 or 4 years of age and might be a cause of relapses. Under the microscope, you can see an increase of collagen fibers and cells in the ligaments of neonates. The bundles of collagen fibers display a wavy appearance known as crimp. This crimp allows the ligaments to be stretched. Gentle stretching of the ligaments in the infant causes no harm. The crimp reappears a few days later, allowing for further stretching. That is why manual correction of the deformity is feasible. Kinematics : The correction of the severe displacements of the tarsal bones in clubfoot requires a clear understanding of the functional anatomy of the tarsus. In the clubfoot the anterior portion of the calcaneus lies beneath the head of the talus. This position causes varus and equinus deformity of the heel. Attempts to push the calcaneus into eversion without abducting it will press the calcaneus against the talus and will not correct the heel varus. Lateral displacement (abduction) of the calcaneus to its normal relationship with the talus will correct the heel varus deformity of the clubfoot. The clubfoot deformity occurs mostly in the tarsus. The tarsal bones, which are mostly made of cartilage, are in the most extreme positions of Vishal Moudgil 92

10 flexion, adduction, and inversion at birth. The talus is in severe plantar flexion, its neck is medially and plantarly deflected, and its head is wedge shaped. The navicular is severely medially displaced, close to the medial malleolus, and articulates with the medial surface of the head of the talus. The calcaneus is adducted and inverted under the talus. The navicular is medially displaced and articulates only with the medial aspect of the head of the talus. The cuneiforms are seen to the right of the navicular, and the cuboid is underneath it. The calcaneocuboid joint is directed posteromedially. The anterior two-third of the calcaneus is seen underneath the talus. The tendons of the tibialis anterior, extensor hallucis longus, and extensor digitorum longus are medially displaced. No single axis of motion (like a mitered hinge) exists on which to rotate the tarsus, whether in a normal or a clubfoot. The tarsal joints are functionally interdependent. The movement of each tarsal bone involves simultaneous shifts in the adjacent bonus. Joint motions are determined by the curvature of the joint surfaces and by the orientation and structure of the binding ligaments. Each joint has its own specific motion pattern. Therefore, correction of the extreme medial displacement and inversion of the tarsal bones in the clubfoot necessitates a simultaneous gradual lateral shift of the navicular, cuboid, and calcaneus before they can be everted into a neutral position. These displacements are feasible because the taut tarsal ligaments can be gradually stretched. Correction of clubfoot is accomplished by abducting the foot in supination while counter pressure is applied over the lateral aspect of the head of the talus to prevent rotation of the talus in the ankle. A well-molded cast maintains the foot in an improved position. The ligaments should never be stretched beyond their natural amount of give After 5 days, the ligaments can be stretched again to further improve the degree of correction of the deformity. The bones and joints remodel with each cast because of the inherent properties of young connective tissue, cartilage, and bone, which respond to the changes in the direction of mechanical stimuli. This has been demonstrated by Pirani, comparing the clinical and magnetic resonance imaging appearance before, during, and at the end of cast treatment. Before treatment, the navcular is displaced to the medial side of the head of the talus. This relationship normalizes during cast treatment. Similarly, the cuboid becomes aligned with the calcaneus during the same cast treatment. Before applying the last plaster cast, the tendo Achillis may have to be percutaneously sectioned to achieve complete correction of the equinus. The tendo Achillis, unlike the tarsal ligaments that are stretchable, is made of non-stretchable, thick, tight collagen bundles with few cells. The last cast is left in place for 3 weeks while the severed Achilles tendon regenerates in the proper length with minimal scarring. At that point, the tarsal joints have remodeled in the corrected positions. In summary, most cases of clubfoot are corrected after five to six cast changes and, in many cases, a tendo Achillis tentomy. This technique results in feet that are strong, flexible, and plantigrade. Definitions of Movements of the Tarsal Bones and of the foot Adduction is the movement of a tarsal bone in which the distal part of this bone moves towards the median body plane; Abduction is the movement in the opposite direction to adduction. Flexion is the movement of a tarsal bone in which the distal part of that bone moves in the plantar direction; Inversion is the movement of a tarsal bone in which the undersurface of the bone moves towards the median body plane; Eversion is the movement in the opposite direction to inversion; Supination is the combined movements of adduction, flexion and inversion; Vishal Moudgil 93

11 Pronation is the combined movements of abduction, extension and eversion; Heel varus results from the movements of inversion and adduction of the calcaneus; Heel valgus results from movements of eversion and abduction of the calcaneus; Forefoot supination is a combined movement of inversion and adduction of the forepart of the foot; Forefoot pronation is a combined movement of eversion and abduction of the forepart of the foot; Equinus is an increase of the plantar flexion of the foot;and Cavus is an increase in the height of the vault of the foot. Pathoanatomy of thecongenitalclubfoot The congenital clubfoot is a complex deformity which clinically has four components : Hindfoot equinus Hindfoot varus Midfoot/forefoot adductus Cavus A clear understanding of the nature and degree of deviations fromnormal in the tarsal and metatarsal elements of the clubfoot is necessary to understand the Ponseti Method of treatment. The most severe deformities occur in the hindfoot. Of the various description of the pathonatomy of the congenital clubfoot, that of Ponseti is the most clear. Ankle joint Severe tibio talar plantar flexion. Talus Medical inclination of neck. Subtalar joint The calcaneus is adducted and inverted under the talus such that the anterior tuberosity of the calcaneus lays under the head of the talus rather than lateral to it. Talonavicular joint The navicular is severely medially displaced, adducted and inverted such that - it articulates only with the medial part of the head of the talus - its medial pole approaches the medial malleolus - the lateral part of the head of the talus is uncovered Calcaneocuboid joint The cuboid is medially displaced and adducted in front of the calcaneus such that only the medial part of the anterior tuberosity of the calcaneus articulated with the cuboid. Naviculo-cuneiform joints The cuneiforms are displaced downward and medially in front of the navicular. The gastricnemius muscle The gastrocnemius muscle of the leg with a clubfoot is shorter than normal. The metatarsals The forefoot, although adducted and inverted, is not as severely inverted as the hindfoot. Hence it is actually pronated with respect to the hindfoot. The first metatarsal is more plantar flexed than the 5th metatarsal, resulting in cavus. THE PONSETI METHOD OF CLUBFOOT TREATMENT General Principles and Aim of Treatment The goal is to reduce if not eliminate all elements of the clubfoot deformity, hence leaving a functional/pain free normal looking plantigrade, mobile, callous free and normally shoeable foot. All components of the clubfoor deformity must be corrected simultaneously, not in sequence, except for equines, which should be corrected last. The cavus, which arises from pronation of the forefoot in relation to the hindfoot is corrected by supinating the forefoot in proper alignment with the hindfoot. With the arch well moulded and the foot in slight supination, the entire foot can be gently and gradually abducted under the talus, which is secured against rotation in the ankle mortise by applying counterpressure with the thumb against the lateral part of the talus. Heel varus will be corrected when the entire foot is fully abducted. Finally, equinus is corrected by dorsiflexing the foot. This is generally facilitated by a simplepercutaneous tenotomy of the tendo Achilles. Vishal Moudgil 94

12 Well moulded plaster casts are applied after manipulations are complete. Details of Ponseti Technique After a general examination to rule out nonidiopathic clubfeet, parents must be reassured that their baby s deformity will be corrected in two months and that the baby will have a normal looking and functional foot throughout life. Radiographs are not necessary. Foot examination and corrective manipulations are best done with the baby resting on the mother s lap. When applying the plaster cast, the baby is placed at one end of the table to provide room for the mother and assistant on either side. A bottle of milk and toys with soothing music relax the baby. 1. Correction of cavus The first element of management is correction of the cavus deformity by positioning the forefoot in proper alignment with the hindfoot. The cavus is corrected as the foot is abducted by supinating the forefoot, which elevates the first ray thereby placing the forefoot in proper alignment with the hindfoot. Since the cavus usually is not a fixed deformity at birth, correction often occurs with the first cast. Correction of severe cavus in a stiff foot will need 2 or 3 cast changes with the forefoot in forced supination. Alignment of the forefoot with the hindfoot to produce a normal arch is necessary for effective abduction of the foot to correct the adductus and varus. 2. Manipulation The manipulation consists of abduction of the foot beneath the stabilized talar head. Locate the head of the talus. All components of clubfoot deformity, except for the ankle equines, are corrected simultaneously. To gain this correction, you must locate the head of the talus, which is the fulcrum for correction. 3. Correction of Adduction and varus Manipulations to correct the varus and adduction: outward pressure is exerted on the metatarsals and counter pressure on the lateral aspect of the head of the talus. This manipulation abducts the foot held in flexion and supination. When the navicular, the cuboid, and the entire foot are displaced laterally in relation to the head of the talus, the anterior portion of the calcaneus follows; thus, the heel varus deformity is corrected. Use gentle continuous pressure for a few seconds. Repeat one or two more times when the baby is relaxed. Do not struggle with the baby. When the foot is abducted, the heel varus will correct as the calcaneus abducts by rotating under the talus. When the calcaneus abducts, it simultaneously extends and everts owing to the curvature profiles of the subtalar joint. The calcaneus cannot everts unless it is abducted. The heel should not be touched. The improvements obtained byeach manipulation are maintained by immobilizing the foot in a w ellmoulded plaster cast for 5 to 7 days. One or two layers of soft roll are applied over the foot and leg. The soft roll as well as the plaster cast that follows should be wrapped snugly over the foot and ankle for better moulding and loosely over the calf and thigh to prevent unnecessary pressure on the muscles. The tips of your thumb and index finger to prevent should cover the toes. The plaster should extend to below the knee first. The plaster is moulded with gentleness and anatomic precision. The heel promience should be emphasized by moulding around it instead of pressing on it. While the foot is held in abduction and supination, the thumb should never rest for long over the lateral aspect of the head of the talus to avoid a dent on the plaster as it sets. The correction is maintained not through pressure but through moulding. At the same time the ankle and malleoli should be gently moulded. In the final manipulation usually done after 4 or 5 casts, the entire foot is abducted between 50 to 60 degrees under the talus and is no longer supinated. The foot should never be everted. The navicular moves laterally away from the medial malleolus to a distance of 1 ½ cm. The lateral aspect of the head of the talus cannot be palpated since it is covered by the navicular. The heel is in some valgus. Vishal Moudgil 95

13 Now the equinus can be corrected by dorsiflexing the foot. The tendo Achilles may need to be percutaneosuly sectioned to facilitate this correction if at least 10 degrees of dorsiflexion cannot be obtained. Ninety percent of babies will require a tenotomy. 4. Tenotomy : Decision to perform tenotomy This decision in determining when sufficient correction has been obtained to perform a percutaneous tenotomy to gain dorsiflexion and to complete the treatment is reached when the anterior calcaneus can be abducted from underneath the talus. This abduction allows the foot to be safely dorsiflexed without crushing the talus between the calcaneus and tibia. If the adequacy of abduction is uncertain, apply another cast or two to be certain. Technique of tenotomy This may be done in the outpatient department. One needs a foot holder and a tenotomist. The foot holder grips the leg below the knee and the midfoot. A generous betadine prep of the heel and ankle is applied, extending medially and laterally. A small amount of local anaesthetic is placed medial to the Achilles tendon 1 cm proximal to its insertion. (Do not use a lot or the tendon is difficult to feel.) Insert the knofe (a number 15 scapel or beaver blade works well) parallel and beneath (anterior) to the tendon approximately one centimeter above its insertion into the calcaneus. Turn the sharp edge of the knife to the tendon and perform the tenotomy. The release obtained after a complete tenotomy is easily felt. Ten to twenty degrees of additional dorsiflexion will be obtained. Clean skin with spirit to remove the betadine. Place asterile dressing over the wound. Wrap with soft roll. Post tenotomy cast Place a well moulded plaster cast with the foot in 20 degrees of dorsiflexion and 70 degrees of abduction (with no eversion). This last cast is worn for 2 ½ to 3 weeks. Many degrees of severity and rigidity of clubfoot are found at birth. Failures in treatment are related more often to faulty technique of manipulation and casting rather than severity of deformity. The Columbian clubfoot score (CCS) can indicate when the foot is ready for a tenotomy. (MFCS<1; HFCS>1). The graph (figure 22) shows the effects of Ponseti treatment and tenotomy on the MFCS, HFCS & TS. 5. Cast removal and bracing protocol : The cast is removed that three weeks and correction is noted. If 30 degree of dorsiflexion is possible, the foot is well corrected, the foot is ready for bracing. Bracing protocol The brace is applied immediately after the last cast is removed, 3 weeks after tenotomy. The brace consists of open toe high-top straight last shoes attached to a bar. For unilateral cases, the brace is set at 75 degrees of external rotation on the clubfoot side and 45 degrees of external rotation on the normal side. In bilateral cases, it is set at 70 degrees of external rotation on each side. The bar should be of sufficient length so that the heels of the shoes are at shoulder width. A common error is to prescribe too short a bar, which the child finds uncomfortable. A narrow brace is a common reason for a lack of compliance. The bar should be bent 5 to 10 degrees with the convexity away from the child, to hold the feet in dorsiflexion. The brace should be worn fulltime (day and night) for the first 3 months after the tenotomy cast is removed. After that, the child should wear the brace for 12 hours at night and 2 to 4 hours in the middle of the day of a total of 14 to 16 hours during each 24-hour period. This protocol continues until the child is 3 to 4 days of age. Types of braces Several types of commercial made braces are available. With some designs, the bar is permanently attached to the bottoms of the shoes. With other designs, it is removable. With some designs, the bar length is adjustable, and with Vishal Moudgil 96

14 others, it is fixed. Steenbeek designed a brace, which is made at a cost of approximately US $ 12. Parents should be given a prescription for a brace at the time of the tenotomy. This gives them 3 weeks to organize themselves. In the United States, the Markell shoe and brace is most commonly used, but other countries have different options. Steen Beeks brace is the most popular because of its low cost and easy manufacturing techniques. Bracing is an essential part of Ponseti management. Unless effective beaces can be provided, the treatment wiil fail. H.M. Steenbeek Cheshire Home in Kampala, Uganda, developed a brace that can be made from simple, easily available materials. The brace is effective in maintaing correction, easy to use, easy to fabricate, inexpensive, and ideally suited for widespread use. What is the Steenbeek Brace? This brace maintains correction in children with treated clubfoot. The brace forms an integral part of Poseti management. This brace has open toe leather shoes with lace closures. A round metal bar holds the shoes in 70 degree of abduction and 10 to 15 degrees of dorsiflexion. The positions of both shoes can be changed toward more or less abduction and dorsiflexion by bending the round bar close to the shoe. The shoe has an inspection hole on the medial side of a well molded heel counter to confirm that the heel is properly placed in the shoe. The heel counter has a low posterior cut (in contrast to high-top shoes) which prevents the heel from slipping out from the brace. There are eight sizes with standardized patterns for all parts, which allows prefabrication and bulk production. This enables the clinician to fit braces off the shelf. These braces are, in most cases, reusable for other children with treated clubfoot. Rationale for bracing At the end of casting, the foot is abducted to an exaggerated amount, which should measure 75 degrees (thigh-foot axis). After the tenotomy, the final cast is left in place for 3 weeks. Ponseti s protocol then called for a brace to maintain the abduction. This is a bar attached to straight last open toe shoes. This degree of foot abduction is required to maintain the abduction of the calcaneus and forefoot and prevent recurrence. The foot will gradually turn back inward, to a point typically to 10 degrees of external rotation. The medial soft tissues stay stretched out only if the brace is used after the casting. In the brace, the knees are left free, so the child can kick them straight to stretch the gastrosoleus tendon. The abduction of the feet in the brace, combined with the slight bend (convexity away from the child), causes the feet to dorsiflex. This helps maintain the stretch on the gastrocnemius muscle and Achilles tendon. Importance of bracing The Ponseti manipulations combined with the percutaneous tenotomy regularly achieve an excellent result. However without a diligent follow-up bracing program, recurrence and relapse occur in more than 80% of cases. This is in contrast to a relapse rate of only 6% in compliant families (Morcuende et al). Total time period for bracing Severe feet should be braced until age 4 years, and mild feet can be braced until age 2 years. It is not always easy to distinguish which foot is mild and which is severe, especially when observing them at age 2 years. Therefore, it is recommended that even the mild feet should be braced for up to 3 to 4 years, provided the child still tolerates the nighttime bracing. Most children get used to the bracing, and it becomes part of their life style. However, if compliance becomes very problematic after age 2 years, if compliance becomes very problematic after age 2 years, it may become necessary to discontinue the bracing to ensure that the child and parents get a good night s sleep. This leniency is not tolerable in the younger age groups. Below age 2 years, the children and their Vishal Moudgil 97

15 families must be encouraged to comply with the bracing protocol at all costs. 6. Managing Relapses : Recognizing relapses After applying the brace for the first time after the tentomy cast is removed, the child returns according to the following suggested schedule. à 2 weeks (to troubleshoot compliance issues) à 3 months (to graduate to the nights-and-naps protocol) à Every 4 months until age 3 years (to monitor compliance and check for replaces) à Every 6 months until age 4 years à Every 1 to 2 years until skeletal maturity Early replaces in the infant show loss of foot abduction and/or loss of dorsiflexion correction and/or recurrence of metatarsus adductus. Relapses in toddlers can be diagnosed by examining the child walking. As the child walks toward the examiner, took for supination of the forefoot, indicating an overpowering tibialis anterior muscle and weak peroneals. As the child walks away from the examiner, look for heel varus. The seated child should be examined for ankle range of motion and loss of passive dorsiflexion. Reasons for relapses The most common cause of replace is noncompliance to the post-tenotomy bracing program. Morcuende found that relapses occur in only 6% of complaint families and more than 80% of noncompliant families. In brace-complaint patients, the basic underlying muscle imbalance of the foot is what causes relapses. Casting for relapses Do not ignore relapses! At the first sign of relapse, consider reapplying one to three casts to stretch the foot out and regain correction. This may appear at first to be a daunting task in a wriggly 14-month-old toddler, but it is important. The casting management is identical to the original Ponseti casting used in infancy. Once the foot is re-corrected with the casts, the bracing program is again begun. Equinus relapse Recurrent equinus is a structural deformity that can complicate management. Equinus can be assessed clinically, but to illustrate the problem, a radiograph is included to show the deformity. Several plaster casts may be needed to correct the equinus to at least a neutral position of the calcancus. Sometimes, it may be necessary to repeat the percutaneous tenotomy in children up to 1 or even 2 years of age. They should undergo casting for 4 weeks postoperatively, with the foot abducted in a long leg bent knee cast, and then go back into the brace at night. In rare situations, open Achilles lengthening may be necessary in the older child. Varus relapse Varus heel relapses are more common than equinus replaces. They can be seen with the child standing and should be treated by re-casting in the child between age 12 and 24 months, followed by reinstitution of a strict bracing protocol. Dynamic supination Some children will require anterior tibialis tendon transfer for dynamic supination deformity, typically between ages 2 and 4 years. Anterior tibialis tendon transfer should be considered only when the deformity is dynamic and no structural deformity exists. Transfers should be delayed until radiographs show ossification of the lateral cuneiform that typically occurs at approximately 30 months of age. Nonnally, bracing is not required after this procedure. One thing is certain: relapses that occur after Ponseti management are easier to deal with than relapses that occur after traditional posteriomedial release surgery. 7. The Recurrent Clubfoot Deformity Early (First and Second Year of life) Recurrence Recurrence is almost always due to failure of brace wear. It is accompanied by a loss of dorsiflexion, followed by the development of heel varus and often some adductus. The cavus rarely recurs. Vishal Moudgil 98

16 The importance of the brace must be explained to the parents. Early recognition is important. IF the foot is not easily dorsiflexed or the child is walking on the outside of the foot (even if the foot is passively correctable), treatment should be instituted. Have the child walk away and towards you. Recurrence may recognized by early heel rise or any heel varus (child walking away) or weight bearing along mainly the lateral ray (child walking towards you). Treatment consists of 2 to 3 more casts at 1 to 2 week intervals. The manipulation is the same. If dorsiflexion is limited, the tendo Achilles tenotomy may be repeated up to one year. (After one year of age anopen TAL (tendo Achilles lengthening) is generally done.) A second recurrence is treated the same way. If recurrence is in the second year of life, the child will often need an anterior tibial tendon trandfer. Repeated casting may not fully correct the foot but will buy time until a TAL and anterior tibial tendon transfer (ATT) to the third cuneiform can be performed. Late Recurrence 2 ½ to 5 Years Old Recurrence is extremely rare after 5 years of age. A dynamic supination may develop at this age. The foot can still be passively positioned with the heel in valgus, but the child supinates the foot in swing phase and is on the outside border of the foot in stance phase. Variable amounts of dorsiflexion/plantarflexion are present. If the foot easily dorsiflexes 10 degrees, an ATT alone is needed. If less dorsiflexion is present, a TAL and rarely posterior release are required. Don t hesitate to recommend ATT in the age group. Long-term follow-up results are as good in patients undergoing ATT as those who did not need it. If the foot is passively correctable, serial casting should be done to get the heel into valgus before the tendon transfer. AFTER TREATMENT WITH PONSETI CAST Infant After successful Ponsetti treatment, +/- percutaneous tenotomy Steenbeek foot abduction brace full time 3mo. Or until walking, then Steenbeek foot abduction brace night time full until 2 yrs. Age Daily stretching exercises by mother. Infant < 2yrs. After soft tissue release (Cincinatti or Turco) POP X 3 months. 6wks AK, 6 wks BK. AFB night splint X 1-2 years Child > 2 yrs After soft tissue release (Cincinatti or Turco) POP X 3 months. 6wks AK, 6 wks BK. AFO walking splint +/- Steenbeek foot abduction brace at night until outgrown Child > 4 yrs After STR and osteotomy POP X 3 months, BK AFO walking splint X 1 until outgrown Child > 4 yrs After tibialis anterior tendon transfer POP X 6 weeks AFO walking splint X 1 until outgrown Adolescent After triple arthrodeses POP X 3 months. No orthoses required The results of treatment are only as good as the post-operative care. Plaster changes need to be bone expertly, maintaing full correction and, in some cases, manipulating the foot further. Common errors in the treatment of the congenital clubfoot : The common errors in the treatment of the clubfoot and how to avoid them are : Pronation of the foot is an error 1. Pronation or eversion of the foot. The wrong assumption is made that the severe supination in the clubfoot with correct by pronating or everting the foot. Pronation of the foot will make the deformity worse by increasing the cavus and locking the adducted calcaneus under the talus, Vishal Moudgil 99