CTEV/ Clubfoot

residency days

1. Congenital Talipes Equinovarus Dr Sijan Bhattachan 2nd year resident NAMS

2. Introduction Probably the most common congenital orthopaedic condition requiring intensive treatment. Represents congenital dysplasia of all musculoskeletal tissues (musculotendinous, ligamentous, osteoarticular and neurovascular structures) distal to the knee Incidence is 1:250 to 1:1000 depending on population Highest prevalence in Hawaiians and Maoris. Males 1/3rd cases are B/L

3. Genetic aetiology is strongly associated Familial occurrence in 25%. Associated conditions Neural tube defects (myelomeningocele and spinal dysraphism) Arthrogryposis Hand anomalies Tibial hemimelia and constriction rings.

4. Many theories on the aetiology have been proposed. Arrest in embryonic development -In normal fetal development of lower limb, the foot in a 6-8 week old foetus has many characterisctics of a congenital clubfoot, including equinus, supination, forefoot adduction and medial deviation of talar neck. -Bohm proposed that an arrest in fetal development at this stage was responsible for the clinical deformities noted at birth.

5. Retractive fibrotic response -Zimny and colleages, identified myofibroblastic retractile tissue in the medial ligaments responsible for innate stiffness of clubfeet. -Increase in collagen fibres and fibroblastic cells in the ligaments and tendons of clubfoot -This hypothesis supported by studies demonstrating abnormal ligamentous and fascial restraints in soft tissues that inherently resist correction of deformity

6. Primary germ plasm defect in the talus causes continued plantar flexion and inversion of this bone, with subsequent soft tissue changes in the joints and musculotendinous complexes Etiology of clubfeet is multifactorial and modulated significantly by developmental aberrations early in limb bud development.

7. Pathoanatomy Muscles contractures lead to the characteristic deformity that includes (CAVE) -Midfoot cavus (tight intrinsics,FHL,FDL) -Forefoot adductus (tight TP) -Hindfoot varus (tight tendoachilles,TP,TA) -Hindfoot equinus (tight tendoachilles)

8. Bony deformity -Talar neck is medially and plantarly deviated, where as the body is rotated slightly outwards in relation to both calcaneum and ankle mortise.; Talar neck body declination angle invariably decreased (approx 90 degrees) as compared to normal 150-160 degrees. -Calcaneus in varus and rotated medially around talus -Navicular and cuboid displaced medially Skin and soft tissues of calf and the medial side of foot are short and underdeveloped

9. Presentation O/E Deformity is usually obvious at birth; the foot is turned and twisted inwards so that the sole faces posteromedially small foot and calf shortened tibia Medial and posterior foot skin creases Heel is higher than the forefoot, which points downwards and inwards.

10. Foot deformities; -Hindfoot in equinus and varus (rigid and resistant to passive correction) -Midfoot in cavus (high medial arch) -Forefoot in adduction In normal baby, the foot can be dorsiflexed and everted until the toes touch the front of leg but in clubfoot, this manoeuvre meets with varying degrees of resistance and in severe cases the deformity is fixed.

11. Lack of correct ability separates a true clubfoot from the milder postural clubfoot. Milder manifestations represent an in utero postural deformity, identified by the fact that it is fully (or nearly fully) correctable passively and by the conspicuous absence of the significant contractures and deep skin creased of a true clubfoot.

12. Infant must always be examined for associated disorders such as DDH and spina bifida.

13. Prognosis for a nonindiopathic syndromic clubfoot is generally worse than that for a idiopathic clubfoot, although there are certain exceptions, such as Down syndrome or Larsen syndrome.

14. Two commonly used classifications by Pirani et al and Dimeglio et al are based solely on physical examination. Pirani’s system is composed of 6 different physical examination findings In Dimeglio’s system, four parameters are assessed on the basis of their reducibility with gentle manipulation as measured with goniometer. Routine clinical use of one or both of these classification systems can be helpful in documenting maintenance of correction or recurrence over time.

15. Pirani severity scoring A reliable method for assessing the amount of deformity formulated by Dr Shafique Pirani, Canada. 6 parameters; 3 of midfoot, and 3 of hindfoot Each parameter is given a value as follows; - 0;Normal - 0.5; Moderately abnormal - 1; Severe

16. MFCS; MEDIAL CREASE Assess the depth of crease and presence of other creases Score Several fine creases Two or three moderate creases 0.5 Deep crease 1

17. CURVED LATERAL BORDER Make sure child’s foot is relaxed Observe from the plantar aspect and use a pen held against edge of foot Assess the point on lateral border of foot at which it deviates from a straight line Score Border straight without deviation Deviates at the level of metatarsals 0.5 Deviates at calcaneocuboid joint 1

18. LATERAL HEAD OF TALUS Palpate the head of talus with foot uncorrected. Keeping finger on talus, correct the foot Score Talus completely sinks away under navicular Talus moves partially but doesn't completely sink 0.5 Talus remains fixed 1

19. HFCS; POSTERIOR CREASE Assess the presence and depth of crease; Posterior Crease Score Several fine crease Two or moderate creases 0.5 Deep crease 1

20. EMPTY HEEL Hold the foot in mild correction and palpate with a single index finger; Assess how much flesh is there between finger and calcaneus Score Easy to palpate calcaneus and not far under skin Palpatable calcaneus which is felt through a layer of flesh 0.5 Calcaneus deep under a layer of tissue and very difficult to palpate 1

21. RIGID EQUINUS Correct the plantar flexion as much as is comfortable for child. Degree of dorsiflexion Score >90 degree 90 degree 0.5 <90 degree 1

22. Uses of Pirani’s score Assessment of progress by serial plotting of score. Predicting need for tenotomy (HFCS>1 and MFCS>1) Estimation of probable number of casts required Very good interobserver reliability and reproducibility.

23. Demeglio’s scoring

24. Radiographs AP (with foot 30 degree plantar flexed and tube likewise angled 30 degree perpendicular) Talocalcaneal (Kite) angle is <20 degree (Normal 20-40 degree); One line through long axis of talus parallel to its medial border and other through calcaneum parallel to its lateral border

25. Talus-1st MT angle is negative (Normal 0-20 degree) indicating adduction of forefoot.

26. Dorsiflexion lateral (Turco view) Talocalcaneal angle <20 degree(Normal 40 degree); Lines drawn through the mid longitudinal axis of the talus and the lower border of the calcaneum.

27. Talocalcaneal angle less than 20 degrees in lateral film shows that the calcaneum cannot be tilted up into true dorsiflexion. The foot may seem to be dorsiflexed but it may actually have broken at the mid tarsal level, producing the so called “Rocker bottom deformity”

28. USG Helpful in prenatal diagnosis Can be diagnosed as early as 12 weeks of gestational age

29. Management The aim of treatment is to produce and maintain a plantigrade, supple foot that will function well. There are several methods of treatment but relapse is common, especially in babies with associated neuromuscular disorders.

30. Non operative Treatment should begin early, preferably within a day or two. Repeated manipulation and adhesive strapping that maintains the correction Serial manipulation and casting (Ponseti method)

31. Operative modalities; Posteromedial soft tissue release and tendon lengthening Medial column lengthening or lateral column shortening osteotomy or cuboid decancellation Triple arthrodesis Talectomy Multiplanar supramalleolar osteotomy Gradual correction by means of ring fixator

32. Serial manipulation and casting Goal is to rotate foot laterally around a fixed talus Order of correction (CAVE) -Midfoot cavus -Forefoot adductus -Hindfoot varus -Hindfoot equinus

33. Ponseti casting technique Most significant event in the history of CTEV Successful correction reported in more than 90% of children 2 yrs and younger. Achilles tenotomy is generally required. Reported recurrence rates range from 10-30% Consists of two phases; Treatment and Maintenance.

34. Treatment phase should begin as early as possible, optimally within the first week of life Gentle manipulation and casting are done weekly The order of correction should be followed. To stretch the ligaments and gradually correct the deformity, the foot is manipulated for 1-3 minutes; Correction is maintained for 5-7 days with a plaster cast extending from the toes to the upper third of thigh and the knee at 90 degrees of flexion Generally five to six casts are required to correct the alignment of the foot and ankle fully. Before application of the final cast, most infants require percutaneous achilles tenotomy to gain adequate lengthening of Achilles tendon and prevent a rocker bottom deformity.

35. First cast application corrects CAVUS deformity by forefoot supination relative to hindfoot, Manipulation seems counterintuitive because it tends to exaggerate the appearance of overall toe inversion. Elevation of first metatarsal and supination of forefoot. Cavus is almost always corrected with the first cast.

36. At successive manipulation and casting sessions, Metatarsus ADDUCTUS and hindfoot VARUS are simultaneously corrected by abducting the foot while counter pressure is applied laterally over the talar head; Calcaneus , navicular and cuboid are gradually displaced laterally. This key maneouvre corrects majority of clubfoot deformity and must be performed at each session with attention to three points; -Forefoot adduction should be performed with the foot in slight supination -Heel should not be constrained by premature dorsiflexion. -Care is taken to locate the fulcrum for counter pressure on the lateral head of talus

37. In general three or four weekly manipulation and casting sessions are required to loosen the medial ligamentous structures of the tarsus and partially mould the joints.

38. EQUINUS is the last deformity that is corrected and correction should be attempted when the hindfoot is in neutral to slight valgus and the foot is abducted 70 degrees relative to the leg Foot is dorsiflexed by applying pressure under the entire sole of the foot and not much under the metatarsal heads to avoid a rocker bottom deformity. To facilitate more rapid correction, subcutaneous heel cord tenotomy is performed in the vast majority of patients.

39. Technique of percutaneous heel cord tenotomy ; After standard sterile preparation, foot is held by an assistant with mild to moderate dorsiflexion pressure. Blade enters the skin along the medial border of achilles tendon. Important to cut the tendon 0.5 to 1 cm proximal to its insertion. After insertion, the blade is pushed medial to the tendon and rotated underneath it. Excessive motion of blade laterally places lesser saphenous vein and sural nerve at risk

40. Successful tenotomy is heralded by a palpable pop and immediate ability for further dorsiflexion of approx 15-20 degrees. No stitches; Sterile cotton cast padding followed by application of long leg cast in maximal dorsiflexion with abduction to 70 degrees and immobilized for 3-4 weeks.

41. Maintenance phase; When final cast is removed, the infant is placed in a brace that maintains the foot in its corrected position (abducted and dorsiflexed) Brace (Foot abduction orthosis) consists of shoes mounted to a bar in a position of 70 degree of external rotation and 15 degree of dorsiflexion.

42. Denis Browne bar and shoes is a foot abduction orthosis prescribed to prevent recurrence of the deformity , to favour remodelling of joints with the bones in proper alignment and to increase leg and foot muscle strength. Orthosis is worn full time for at least 3-4 months and afterwards it is worn at nap and nighttime for 2-4 years Every effort should be made to assist families for its consistent use because lack of compliance with brace wear is the primary reason for recurrence of deformity and failure of this treatment method

43. The objective is to achieve not only correction but overcorrection. The position should be checked by xray in order to ensure that there is no rocker bottom defect; attempts to overcome equines before the other deformities are corrected may break the foot in the mid tarsal region. Resistant cases will usually declare themselves after 8-12 weeks of serial manipulation and strapping.

44. Early recurrence of deformity (within 1 year) may be successfully salvaged in one third of relapsed cases by repeat manipulation and casting to stretch and correct any residual deformity. Approx two third of feet that relapse, do not respond sufficiently to repeat casting and ultimately require surgical intervention

45. Operative treatment The objectives of clubfoot surgery are; -the complete release of joint ‘tethers’ (capsular and ligamentous contractors and fibrotic bands) -lengthening of tendons so that the foot can be positioned normally without undue tension

46. Approaches Extended posteromedial incision (Turco) Posterior curved transverse incision extended anteriorly on both medial and lateral sides (Cincinatti) Posterolateral incision combined with a separate curved medial incision (Caroll)

47. Transverse circumferential ( Cincinnati ) incision is preferred. Begin incision on the medial aspect of foot in the region on naviculocuneiform joint Carry incision posteriorly to pass transversely over achilles tendon at the level of tibiotalar joint Continue incision in a gentle curve over the lateral malleolus and end it just distal to sinus tarsi

48. Caroll two incision technique

49. Turco incision

50. PMSTR and tendon lengthening Indications Resistant feet in young children Rocker bottom feet that develop as a result of serial casting syndrome associated clubfoot Delayed presentation >1-2 yrs of age Performed at 9-10 months of age so the child can be ambulatory at one year of age.

51. TendoAchilles and tibialis posterior tendons are lengthened through Z divisions; Posterior capsule of ankle and subtalar joints often have to be divided to allow adequate correction of hindfoot equinus. Calcaneo fibular ligament, a key structure in keeping the calcaneus malrotated, is then released. Correction of forefoot deformity is carried out by releasing the contractures around the talonavicular and calcaneocuboid joints Finally origin of intrinsic muscles and plantar fascia from calcaneum may need to be divided to reduce any cavus or plantaris deformity.

52. Complications Loss of correction Dorsal subluxation of navicular Valgus overcorrection Dorsal bunion

53. Modified McKay procedure (Extensile posteromedial and posterolateral release) through a Cincinnati incision is a preferred technique for the initial operative management of most clubfeet. Allows correction of internal rotational deformity of calcaneus and release of contractures of posterolateral and posteromedial foot.

54. Revision or secondary procedures Prevalence of repeat surgery after the initial soft tissue release in infancy is estimated to be in the range of 10%. In selecting revision procedures, the surgeon must strongly consider the inevitable additional stiffness and muscle weakness that result from repeat surgery and immobilization and thus the primary goal of additional procedures must be to achieve the eventual realistic foot position with the least possible number of procedures

55. Soft tissue surgery Anterior tibial tendon transfer; Indicated when there is dynamic inversion or supination of the midfoot, Split transfer ( lateral arm reinserted to cuboid or lateral cuneiform) or entire tendon transfer (inserted to 3rd cuneiform)

56. Transfer for insufficient triceps surae (Calcaneus gait) Overlengthening of achilles tendon or triceps insufficiency secondary to inadequate excursion from scarring is notoriously difficult to reconstruct and is best prevented rather than reconstructed. Muscles available for transfer to reconstruct the triceps achilles complex include Peroneals, Tibialis posterior and long toe flexors.

57. Bony surgery; Lateral column shortening Evans described the use of a wedge resection of calcaneocuboid joint to shorten the lateral column as a part of treatment of relapsed deformity. This approach, which combines posteromedial release and lateral column shortening in one stage, is probably the most common procedure for recurrent clubfoot.

58. Evans procedure has become a standard technique for recurrent clubfoot deformity in which the midfoot is clearly in varus as a result of talonavicular and calcaneocuboid medial displacement. Procedure of choice between 4 and 8 years old.

59. Litchblau operation(simple resection of anterior end of calcaneum) for children <4 yrs old

60. Different levels of osteotomy 1.Vertical osteotomy of anterior part of calcaneum 2. Excision of anterior end of calcaneum (Litchblau procedure) 3. Excision of calcaneocuboid joint and fusion (Evans procedure) 4. Wedge resection and enucleation of cuboid bone.

61. Calcaneal osteotomy In a foot with fixed heel varus, with or without other significant residual deformity, an opening or closing wedge osteotomy or a lateral displacement osteotomy can be used. Advantage of this osteotomy as proposed by Dwyer , is that subtalar motion is preserved.

62. Dwyer technique Skin incision perpedicular to previous incision. Calcaneus is osteotomized roughly parallel to the subtalar joint.

63. Opening wedge Tricortical iliac crest graft recommended

64. Closing wedge.

65. Most authors now prefer lateral closing wedge osteotomy with K wire fixation if necessary. Ideal age for this operation is 3-4 years but there is no upper age limit.

66. Triple arthrodesis Salvage procedure for uncorrected clubfoot in older children and adolescents Corrects the severely deformed foot by a lateral closing wedge osteotomy through subtalar and mid tarsal joints. Indicated in refractory clubfoot at 8-10 yrs of age Contraindicated in insensate foot due to rigidity and resultant ulceration. The distorted anatomy makes triple arthrodesis a real challenge but it is possible to end up with a plantigrade, stable and panfry foot.

67. Talectomy is a salvage procedure in older children (8-10 yrs) with an insensate foot. Multiplanar supramalleolar osteotomy indicated as a salvage procedure in older children with complex, rigid, multiplayer clubfoot deformities that have failed conventional operative management

68. Gradual correction by means of ring fixator indicated in complex deformity resistant to standard methods of treatment; Also helps to achieve lengthening. Ilizarov method has gained popularity. Full correction can be achieved even in feet severely scarred from previous surgery and there is often an increase in the size of foot, which is thought to be due to an increase in blood supply through distraction.

69. After successful correction of deformity, relapses may be prevented by using Dennis Browne boots in infants or moulded ankle foot orthoses in older children

70. Complications Related to nonoperative treatment Deformity relapse Related to surgical treatment Residual cavus Pes planus Undercorrection Intoeing gait Osteonecrosis of talus Dorsal bunion

71. Dorsal bunion that develop after clubfoot surgery has been attributed to muscle weakness, particularly of triceps sure, where in a bunion develops as the patient tries to push off with the toe flexors to compensate for the weakness of triceps or imbalance between the anterior tibial muscle and impaired peroneus muslce Most authors recommend transfer of FHL to neck of first MT combined with bony correction by plantar closing wedge osteotomy of first MT

72. Banskota B 1 , Banskota AK , Regmi R , Rajbhandary T , Shrestha OP , Spiegel DA The Ponseti method in the treatment of children with idiopathic clubfoot presenting between five and ten years of age. Bone Joint J. 2013 Dec;95-B(12):1721-5. doi: 10.1302/0301-620X.95B12.32173. Abstract Our goal was to evaluate the use of Ponseti's method, with minor adaptations, in the treatment of idiopathic clubfeet presenting in children between five and ten years of age. A retrospective review was performed in 36 children (55 feet) with a mean age of 7.4 years (5 to 10), supplemented by digital images and video recordings of gait. There were 19 males and 17 females. The mean follow-up was 31.5 months (24 to 40). The mean number of casts was 9.5 (6 to 11), and all children required surgery , including a percutaneous tenotomy or open tendo Achillis lengthening (49%), posterior release (34.5%), posterior medial soft-tissue release (14.5%), or soft-tissue release combined with an osteotomy (2%) . The mean dorsiflexion of the ankle was 9° (0° to 15°). Forefoot alignment was neutral in 28 feet (51%) or adducted (< 10°) in 20 feet (36%), > 10° in seven feet (13%). Hindfoot alignment was neutral or mild valgus in 26 feet (47%), mild varus (< 10°) in 19 feet (35%), and varus (> 10°) in ten feet (18%). Heel-toe gait was present in 38 feet (86%), and 12 (28%) exhibited weight-bearing on the lateral border (out of a total of 44 feet with gait videos available for analysis). Overt relapse was identified in nine feet (16%, six children). The parents of 27 children (75%) were completely satisfied. A plantigrade foot was achieved in 46 feet (84%) without an extensive soft-tissue release or bony procedure , although under-correction was common, and longer-term follow-up will be required to assess the outcome.

73. References Tachdijan Pediatric orthopaedics, 5th edition Apley’s system of orthopaedics and fractures, 9th edition. Campbell operative orthopaedics,12th edition. orthobullets.com Internet sources