Rod Hammett Consultant Orthopaedic Surgeon Musgrove Park Hospital

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1 Rod Hammett Consultant Orthopaedic Surgeon Musgrove Park Hospital

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6 What patients does the surgeon want to see? What patients does the neurologist want the surgeon to see?? What does the surgeon need to know??? What does the patient want????

7 CMT commonly presents to orthopaedic surgeons with lower and upper limb symptoms including ankle sprains, cavus feet, and weakness of intrinsic hand muscles. More rarely, they present with spinal and hip manifestations. It is not usually a life threatening or painful condition and therefore lends itself to the FRCS (Tr & Orth) exam.

8 Ankle and hindfoot Instability, recurrent sprains Peroneal tendon pain Insertional Achilles problems/plantar fasciopathy Secondary degenerative joint disease Tibial stress syndrome Knee pain/genu varum/itb syn

9 Mid and Forefoot 5th metatarsal Fracture(s) Non union Basal overload Metatarsalgia Midfoot OA Claw toes

10 Neuropathic ulceration Shoe breakdown Diffuse/ multiple complaints Any combination of the above Beware pain fibre neuropathy

11 Neuromuscular until otherwise proven

12 Neuromuscular Peripheral neuropathy Spinal Cord Disease CNS disease Myopathy Congenital Arthrogryposis Residual Club Foot deformity Trauma Post compartment syndrome Peroneal nerve palsy Idiopathic CMT (HMSN) Poliomyelitis/ neoplasia /syringomyelia Freidrich s ataxia, cerebral palsy Muscular dystrophy

13 70% have neurological diagnosis CMT (HMSN) 50% of patients 30% Idiopathic Importance of full neurological assessment Especially FH, bilateral, gait impairment / muscle atrophy Sensory / motor disturbance

14 Described 1886 Spectrum of genetic disorders that affects peripheral nerves Slows action potential transmission or amplitude Usually present with Lower limb Cavus feet / ankle sprains Upper limb Intrinsic weakness

15 Strong genetic component FH (spontaneous genetic mutation) Lots of subtypes of CMT Inheritance pattern What part of nerve cell is affected Genes affect nerve proteins Progressive disease Management Maintain mobility and independence

16 Lower limb pain 90% Rubbing toes 60% Metatarsalgia 50% Ankle instability 30% Lateral border pain 10% Ankle/hindfoot pain 10% Ulceration 1%

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18 Pes cavus Latin hole / pit hollow foot A foot with a high arch that fails to flatten with weight bearing Prevalence 10%

19 Cavovarus Forefoot plantarflexed Hindfoot varus (1 st ray > others pronated) Calcaneocavus Dorsiflexion of calcaneus Fixed equinus of forefoot (HSMN) (POLIO) No pronation of forefoot therefore no varus deformity

20 Plantaris Plantarflexion of forefoot Calcaneus Dorsiflexion of hindfoot Cavovarus Varus deformity of hindfoot

21 Cavus Equino Cavo- Varus Calcaneo- Cavo -Varus

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24 A Meary s Angle N = 0 5 Degrees B Calcaneal Pitch Angle N = 30 degrees C Hibbs Angle N = <45 degrees D Weight Bearing Tibioplantar Angle N = 90 degrees

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30 Peroneus longus (1 st MT and adj cuneiform) Plantarflexion first ray / evertor Tibialis anterior (1 st MT and adj cuneiform) Dorsiflexion Peroneus Brevis (5th MT) Evertor Tibialis posterior (Navicular tuberosity) Invertor / Flexor Intrinsics Flex the MTP / extend IP

31 Posterior muscles normal Anterior weak (variable) Weak Tib Ant Relatively strong Peroneus Longus Forefoot equinus Peronus Brevis weak / Tib Post normal Hindfoot varus

32 Hypothesis Normal anatomy and function Intrinsic and extrinsic muscle balance Acting on normal soft tissues and bones / joints Muscular imbalance Deformity and impaired function

33 Disorder differentially affects extrinsic muscles CONTROVERSIAL Tib post >peroneus brevis Adduction of forefoot and inversion of hindfoot Peroneus longus > Tib ant Plantar flexion of first metatarsal Tib ant weak EHL and EDL aid dorsiflexion MTP dorsiflexion Tib ant> gastrocnemius / soleus (Polio)

34 Disorder affects foot Intrinsic muscles CONTROVERSIAL (Deformity can pre-date extrinsic weakness + MRI findings) Long extensors - Dorsiflexion of MTP Long flexors IP joint flexion Intrinsic shortening + windlass - Plantar aponeurosis tensioned - shortening of medial longitudinal arch Cavus can progress without clawing!

35 Theory 1 and 2 are correct Probably not a single problem in all cavus feet

36 Windlass effect Plantar plates of MTP attached to plantar fascia MTP extension tightens plantar fascia Increase arch - cavus Medial band largest medial calcaneus - varus

37 Tripod effect Heel, 1 st MT head, 5th MT head Plantigrade / no varus or valgus 1 st ray plantarflexed 3 points can only rest on ground if heel varus (Coleman 1977 block test )

38 Plunger effect (Stainsby) MTP extension forces MT heads plantarward MT plantarflexion Bony Deformity Bone growth affected by deforming forces Altered bone shape

39 Variable Toes PIP rubbing / tips of claw toes Metatarsal Heads Metatarsalgia

40 Hindfoot varus Ankle instability (peroneus brevis) Lateral foot pain Stiff hindfoot Unable to absorb forces Fatigue Ankle / hindfoot pain

41 General Shoe fitting /wear Non specific foot pain (50% of CMT not responsive to surgical measures) Weakness Ulceration

42 Most autosomal dominant Demyelination of nerves Presents in second decade Diagnosed by decreased nerve conduction velocities

43 Weakness Ataxia Distal sensory loss Areflexia Equinus/Calcaneus Cavus Varus

44 Autosomal Dominant Neuronal form Later presentation Mildly Impaired Nerve Conduction Velocities

45 Onset in 20s and 30s Profound limb weakness As Type I or Stork leg appearance Flail foot Calcaneo varus or Flat foot

46 Autosomal recessive Includes Dejerine Scottas syndrome (hypertrophic neuropathy of infancy) Presents in infants Demyelination with decreased conduction Sensory loss (Glove and stocking) Muscle weakness and contracture Spinal deformity common

47 Type IV Refsum disease Type V - Inherited spastic paraplegia with distal weakness Type VI - Peroneal muscular atrophy with optic atrophy Type VII Atrophic muscle weakness with retinitis pigmentosa

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55 Explanation Reassurance Natural history & inheritance Neurology/Genetics

56 Orthoses Insoles Metatarsalgia, accomodative, FFO Total contact insole Shoes Lateral wedge & flare Extra depth toebox AFO, ankle brace, caliper

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59 Put the Foot below the leg Put the Foot square to the ground BALANCE THE MUSCLE POWERS

60 À la carte approach Stepwise correction starting proximally and working distally Balance the forces to maintain bony correction (even with fusions)

61 Flexible deformities tendon transfers +/- axial realignment Fixed deformities osteotomies and soft tissue releases Degenerative joint disease arthrodesis (or replacement)

62 Patients with Charcot Marie Tooth have neuropathic pain Surgery may introduce or exacerbate pain Treat structural problems, not pain After Surgery Change the shoes!

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64 Careful examination and weightbearing XR Supramalleolar osteotomy? Achilles lengthening Avoid over lengthening Isolated gastroc?

65 ATFL reconstruction Peroneal tendons Brevis to longus tenodesis

66 Corrects calcaneal pitch Not for plantaris correction Beware medial and lateral plantar nerves

67 Chevron or Z Correction of calcaneus deformity Minimally invasive surgery possible

68 Stiff subtalar/chopart Symptomatic DJD Paralytic deformity Peroneal LMN polio

69 Calcaneal osteotomy 1 st MT osteotomy Gastroc/ Achilles release Tendon transfers

70 Soft tissue releases Adjunct procedures Talectomy Wedge resections Frame

71 Severe cavus Dorsal bump

72 Foot realignment Ankle fusion TTC/ pantalar fusion Ankle replacement

73 Foot realignment Ankle fusion TTC/ pantalar fusion Ankle replacement?

74 Foot realignment Ankle fusion TTC/ pantalar fusion Ankle replacement

75 Three components Cavus Adductus Pronation First ray Composite midfoot (wedge) osteotomies Triple fusion Chopart s joints correct pronation

76 Coleman block test Correctable hindfoot driven by 1 st MT plantarflexion

77 Plunger effect Windlass effect Peroneus longus> tibialis anterior Secondary fixed contracture

78 Treat claw hallux Transfer EHL MT neck > tib ant>lateral Elevate 1 st MT dorsal closing wedge TMT fusion Longus to brevis transfer?

79 Plan from Coleman block test

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81 Restore dynamic balance for correctable deformities Prevent recurrence after osteotomy or arthrodesis

82 Invertors and flexors overpower Extensors and evertors Tibialis posterior and triceps surae overpower Tibialis anterior and peroneus brevis

83 Tibialis posterior Peroneus longus to brevis Tibialis anterior laterally Jones procedure

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85 PF Flexors & intrinsics

86 PF Flexors

87 Dorsal callus Dorsal ulceration Nail walking and tip callous Plunger effect metatarsalgia

88 Type 1 Flexible MTP and IPJ Rarely present Type 2 Stiff IPJ flexible MTP (+/- subluxation) Type 3 Stiff IPJ &MTP

89 Type 1 FETT MTP capsulotomy PIP arthroplasty

90 Type 2 PIP fusion/arthroplasty +/- MTP release

91 Type 3 PIP fusion/arthroplasty +/- radical MTP release & Kwire Or Stainsby hemiphalangectomy

92 Flexible Rarely present FHL transfer to P1

93 Stiff Jones procedure IPJ fusion EHL transfer to MT

94 History Establish patient s problem Treat the patient not x-ray Simple non-operative Mx first

95 Forefoot balancing if forefoot driven Osteotomy and tendon transfer for mobile hindfoot joints Fusion for fixed deformities/oa/paralysis

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