Case Study VI Jocy

1. Case Study VIJocy JONI WILLIAMSON, SPT STACY MURPHY, SPT APRIL 16, 2009 PT 7336

2. Full-term Infant: • Mother surprised to see newborn with inwardly turned left foot after delivery • All prenatal tests were negative for birth defects • Mother referred to orthopedist for equinovarus foot

3. Examination • APGAR • Score = 8 • Observations: • Left equinovarus foot • No other signs of deformity or delay

4. Talipes Equinovarus (Club foot)1 • A deformity of the foot involving also the entire lower leg. a) Congenital - usually an isolated abnormality b) Teratological - usually associated with a neuromuscular disorder (AMC or syndrome complex) • Associations with club foot can include: • Oligohydramnios - deficiency of amniotic fluid • Congenital constriction rings - amniotic band syndrome • Unknown underlying pathology • It is either positional (normal foot that has been held in a deformed position in the uterus) or fixed.

5. More specifically1… • Whole foot = extreme supination • Fore part of the foot • Pronated with respect to the hindfoot, as a result of the cavus deformity • Cavus deformity = the first metatarsal is more plantar flexed than the fifth metatarsal • Navicular and the cuboid • Rotated medially in relation to the talus • Are held in adduction and inversion by contracted ligaments and tendons

6. More specifically1… • Tibial-navicular interval: • Distance between the medial malleolus and the tuberosity of the navicular - shorter intervals indicate worse deformity • The degree of resistance of the navicular to be moved away from the medial malleolus = correlates with the severity of the deformity. • In severe clubfoot, complete reduction of the extreme medial displacement of the navicular may not be possible by manipulation

7. Etiology 2,3: • Exact genetic mechanism of inheritance of congenital talipes equinovarus (CTEV) has been extensively investigated using family studies and other epidemiological methods • The cause of congenital clubfoot is unknown and most infants who have clubfoot have no identifiable cause.

8. Etiology Continued 2,3: • Theories include: • The presence of a number of inheritance patterns • Many different etiologies presenting as the same morphological condition • Complex gene-environment interactions • Genetic associations: • Diastrophic dwarfism • Some syndromes involving chromosomal deletion • Autosomal recessive pattern of clubfoot inheritance

9. Prevalence2 • 1-3/1,000 live births • 2:1 Male>Female • Varies in ethnic groups: • Highest in Polynesian ancestry (7/1,ooo) • Lowest in Asian populations (.57/1,000) • Clubfoot is bilateral in 30%-50% of cases

10. Foot Dysmorphology Diméglio grading system I-IV4

11. Risk Factors5 • Unknown • Limb deformities can be precursor to underlying pathologies • Post-natal detection: • Survival is low and determined by associated anomalies • Pre-natal detection: • earlier and less complicated postnatal surgery • a shorter admission time

12. NCMRR Disability Model • Pathophysiology • Talipes Equinovarus • Impairment • Decreased LE ROM and future weight-bearing status • Unable to meet developmental milestones if not corrected

13. NCMRR Disability Model • Functional Limitation • Will be unable to functionally use lower extremity unless the equinovarus is corrected • Disability • Unable to excel in gross motor function and play with peers • Societal Limitation • If unable to walk or run, Jocy will not be as willingly accepted by her classmates

14. APTA Practice Pattern • Musculoskeletal 4B: Impaired Posture • Musculoskeletal 4C: Impaired Muscle Performance • Musculoskeletal 4D: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Connective Tissue Dysfunction • Musculoskeletal 4I: Impaired Joint Mobility, Motor Function, Muscle Performance, and Range of Motion Associated with Bony or Soft Tissue Surgery

15. Prognosis1 • Good • Multidisciplinary intervention (PT, Orthopedist) • Early detection and treatment • Jocy’s familial support and concern will facilitate improvement in her treatments and/or surgeries

16. PT Goals • LTG: Pt to achieve neutral ankle inversion in 3 months with casting in order to begin correcting ankle dorsiflexion to achieve efficient crawl. • STG: Pt to achieve 10° ankle inversion in 1.5 months with casting.

17. PT Goals • LTG: Pt to achieve 10˚ dorsiflexion in left LE in 6 months in order to pull to stand. • STG: Pt to achieve neutral ankle dorsiflexion in left LE in 4 months.

18. Family Questions? • What are the treatment options? • Why did prenatal tests not indicate deformity before birth? • How will this condition affect child’s growth and motor development?

19. Treatment • Casting • Ponsetti • Surgery • Soft Tissue Release • Percutaneous /Open Tenotomyof the Achilles tendon • Ilizarov Method • Physical Therapy

20. Treatment1 • Ponsetti Serial Casting: • correction of cavus • correction of adduction and heel varus • correction of equinus

21. Ponsetti Serial Casting1 • Begins in 1st week of life to take advantage of the initial elasticity of contracted ligaments, joint capsules and tendons • Within the first 2-3 months : 5-6 manipulation and cast applications • Toe-to-groin plaster casts worn for 5-7 days • Total duration of treatment should be less than 3 months • Children who present for treatment after 4 or 5 months old may require operative correction because ligaments become stiffer

22. Ponsetti Serial Casting1… • Correction of Cavus: • Must be corrected prior to other deformities • Forefoot is supinated and the first metatarsal is dorsiflexed • Correction of Adduction and Heel Varus: • Goal is to abduct the supinated foot under the talus • Cast is placed with knee flexed at 90˚ and foot in maximum external rotation

23. Ponsetti Serial Casting1 • Correction of Equinus: • Dorsiflexing the fully abducted foot to stretch the tight posterior capsules and ligaments of ankle and subtalar joints and the Achilles tendon • If foot is dorsiflexed prior to correction of the hind foot varus, rocker bottom foot may be created

24. Ponsetti Serial Casting1 • If the Achilles tendon remains tight a percutaneous tenotomy will be performed. • A final cast is worn for 3 months to allow the tendon to heal on its own. • A external rotation brace is worn full-time for 3 months and at night until the child is 2 years old.

25. If compliance is an issue for Ponsetti method…4 • Still unknown if parents are noncompliant 2° slip- page and blisters, or if blisters are caused by non- compliance • Flexible braces found to be as effective (post-Ponsetti) • Increase compliance

26. Orthosis: Dynamic KAFO Dennis-Browne Bar Splint Standard Foot Abduction Brace, Open Toe

27. Before Ponsetti4

28. 3 Years after Ponsetti4

29. Surgery6,7 Soft Tissue Release Open Tenotomy of Achilles Tendon Ilizarov Method

30. Surgery6 Talectomy = removal of the talus 1° or salvage sx?? Remember: only 20% of infants require sx 76% chance of relapse in surgically corrected feet

31. Current Management8: • Moving away from operative treatments towards a more conservative treatment using the Ponsetti regime • Boehm’s 2008 study had 12 infants (24 distal arthropyotic feet) participate in Ponsetti method before 6 mo of age • All 24 had standard percutaneus Achilles tendon tenotomy • 22/24 feet fully corrected without further surgery • Idiopathic clubfoot is easier to treat than arthrogryopotic feet

32. Physical Therapy Flexibility exercises: Stretching Mobilization of talonavicular joint Range of motion Balance and coordination: Proprioception Neuromuscular education/re-education Motor function training Weight bearing: Strengthening muscle imbalance Gait training

33. Physical Therapy • Orthotic Devices: • Casting • Bracing • Patient/Caregiver Education: • Home exercise program: • Stretching • Mobilization • Compliance • Patient outcomes/prognosis

34. Evidence9 • “Conservative treatment of clubfoot: the Functional Method and its long-term follow-up” • Paris, France – 2006 • Functional Method: • Consecutive gentle manipulations • 30 minutes per foot daily for 2 weeks after birth • Then 5x week, and decreased progressively to 2 sessions a week • Start with gentle joint distractions, then progressive reduction of each deformity with no counter pressure on bone or cartilaginous frames • Last step is active rehabilitation – muscle imbalance • Flexible splint is applied between sessions of manipulations

35. Evidence9 • 3 wide series published as a sample of the Functional Method protocol • 600 clubfeet in first, 338 in second, 350 in third • All cases regardless of severity were included • The rate of excellent-good functional results improved from 48% to 77% of the cases

36. BUT WHY DID THE PRENATAL TESTS NOT DETECT THIS????

37. Club Foot in Utero

38. Why did the prenatal tests not find the deformity?10 Table III. The earliest week of gestation at which ultrasonographic diagnosis of club foot was made.

39. Videos • Clubfeet without treatment: • http://www.youtube.com/watch?v=NZwzB72aAyo&feature=related • Bracing: • http://www.youtube.com/watch?v=JmaiSkDSBFY&feature=related • After Ponsetti: • http://www.youtube.com/watch?v=bgCVYjE59rY

40. References • Wheeless CR III, MD. Wheeless’ Textbook of Orthopedics. Data Trace Internet Publishing, LLC: 1996. • de Alwis, de Silva, Bandara, Gamage. Prevalence of talipes equinovarus, congenital dislocation of the hip, cleft lip, Down Syndrome and neural tube defects among live newborns in Anuradhapura, Sri Lanka. Sri Lanka Journal of Child Health. 2007; 36: 130-132. • Chesney D, Barker S, Miedzybrodzka Z, Haites N, Maffullini N. Epidemiology (etiology) and genetic theories in the etiology of congenital talipes equinovarus. Hospital for Joint Diseases. 1999;58:59-64. • Boehm S, Limpaphayom N, Alaee F, Dobbs S, Dobbs MB. Early results of the Ponsetti method for the treatment of clubfoot in distal arthrogryposis. J Bone Joint Surg Am. 2008; 90: 1501-1507. • Cohen-Overbeek TE, Grijseels EWM, Lammerink EAG, Hop WCJ, Wladimiroff JW, Diepstraten AFM. Congenital talipes equinovarus: Comparison of outcome between a prenatal diagnosis and a diagnosis after delivery. Prenatal Diagnoses. 2006;26:1248-1253. • Legaspi J, Li YH, Chow W, Leong JC. Talectomy in patients with recurrent deformity in club foot. A long-term follow-up study. J Bone Joint Surg Br. 2001;83:384-387. • Widmann RF, Do TT, Burke SW. Radical soft-tissue release of the arthrogrypotic clubfoot. J Pediatr Orthop B. 2005;14:114-115. • Uglow MG, Clarke NMP. Relapse in staged surgery for congenital talipes eqinovarus. J Bone Joint Surg Br. 2000;82:739-743 • Bensahel H, Jehanno P, Delaby JP, Themar-Noel C. Conservative treatment of clubfoot: the Functional Method and its long-term follow-up. Acta Orthop Traumatol Turc. 2006;40(2):181-186. • Keret D, Ezra E, Lokiec F, Hayek S, Segev E, Wientroub S. Efficacy of prenatal ultrasonography in confirmed club foot. Journal of Bone and Joint Surgery. 2002;84: 1015-1019.

41. Any Questions???