Presentation of cerebral palsy

Orthopaedic management principles of CP

1. Chairperson Dr. R. S. Jatti Presenter Dr. Srinath Gupta Cerebral Palsy

2. Brain Development Brain grossly differentiates into cerebrum and cerebellum during 1 st Trimester of embryonic life Neurons begin to develop in 2 nd trimester By end of 2 nd trimester all neurons are formed and any damage occurring now is irreversible Synaptic connections occur in 3 rd trimester

3. First described in 1862, by William John Little, an orthopedic surgeon who observed that children with tone and developmental abnormalities often had prolonged labor, prematurity or breech delivery Cerebral Palsy was known as Little’s Disease for decades. The term cerebral Palsy originated with William Osler and Sigmund Freud Static Encephalopathy has been used interchangeably with cerebral palsy Trivia

4. Overview Definition Incidence Etiology Classification Management Lower Limb Deformities

5. Definition Static, non progressive disorder of CNS secondary to an insult to immature brain , resulting in varying degrees of motor milestone delay and dysfunction CP is a disorder of tone, posture or movement It results in paralysis, weakness, in coordination or abnormal movement

6. Incidence 2.4-2.7 for every 1000 live births

7. Etiology Prenatal: Infection : TORCH Complex, HIV Cerebral malformation Obstetrical complication : pre- eclampsia , eclampsia , abruptio placentae , placenta previa , placental infarction Maternal diseases Abuse of drugs

8. Contd… Perinatal: Prematurity Low birth weight Complicated delivery Asphyxia Cerebral trauma Hyperbilirubinemia Blood incompatibilities Infections: Herpes simplex, meningitis Severe hypoglycemia

9. Contd… Post natal : Infections : Meningitis, encephalitis Head injury Cerebral anoxia Aspiration Asphyxia Seizures Near drowning Cardiac arrest Cerebrovascular accidents Sickle cell anaemia Vascular malformations

10. Classification Because of the wide variability in presentation and types of cerebral palsy, there is no universally accepted classification scheme.

11. Monoplegia Hemiplegia Paraplegia Diplegia Quadriplegia Double Hemiplegia Total body Classification based on Geographical Distribution

12. Physiological classification Spastic Athetoid Choreiform Rigid Ataxic Hypotonic Mixed

13. Spastic: Most common type Associated with injury to pyramidal tracts in immature brain Athetoid: Associated with injury to extrapyramidal tracts Dyskinetic purposeless movements Dystonia or hypotonia can occur with athetoid cerebral palsy Choreiform: Continual purposeless movements of wrists,fingers,toes and ankles Contd…

14. Rigid: Most hypertonic form Cogwheel or leadpipe rigidity Ataxic: Very rare Injury to developing cerebellum Disturbance of coordinated movement viz. walking Characterized by weakness, in-coordination, a wide based gait , and trouble with fine and rapid movements.  Hypotonic: Passing stage in spastic or ataxic cerebral palsy Mixed: Signs of pyramidal and extrapyramidal deficits Contd…

15. Spastic Diplegia Bilateral spasticity of legs 1 st noticed when infant begins to crawl-tends to drag the legs behind more ( commando crawl) Severe spasticity –application of diaper is difficult due to excess adduction of hips Brisk reflexes, ankle clonus Scissoring posture of lower extremity when suspended by axilla Walking tiptoes, disuse atropy ,impaired growth of lower extremity Intellectual development normal Minimal seizures CT/MRI- periventricular leukomalacia of white matter mainly lower limb fibres All spastic types characterized by toe walking, a crouched gait, and flexed knee, scissoring

16. Spastic Hemiplegia Arms often more involved than leg-difficulty in hand manipulation is obvious by 1 yr Delayed walking -18-24 months Equinovarus deformity of foot, walks on tip toes because of increased tone Affected upper limb has dystonic posture when child runs Deep tendon reflexes increased, ankle clonus , Babinski sign + 1/3 rd have seizure disorder 25% have MR CT/MRI- atrophic cerebral hemisphere with dilated lateral ventricle contralateral to the affected side

17. Spastic Quadreplegia Most severe form ,most common All extremities severely impaired High association with MR and seizures Flexion Contractures of knees and elbows

18. After age 1yr – athetoid movements become evident Speech is affected (slurred, voice modulation impaired) due to involvement of oropharyngeal muscles Upper motor neuron signs –not present Seizure uncommon Intellect –preserved Characterized by an exaggerated step, hip and knee hyperextension, a backwards lean, and shoulder girdle and trunk extension. Athetoid Cerebral palsy

19. I Has nearly normal gross motor function II Walks independently, but has limitations with running and jumping III Uses assistive devices to walk and wheelchair for long distances IV Has ability to stand for transfers, but minimal walking ability; depends on wheelchair for mobility V Lacks head control, can’t sit independently, is dependent for all aspects of care Gross Motor Function Classification System

20. Associated Problems Mental Retardation Communication Disorders Behavioral disorder Seizures Vision Disorders Hearing loss Somatosensation (skin sensation, body awareness) Temperature instability Nutrition Drooling Dentition problems Neurogenic bladder Neurogenic bowel Gastroesophageal reflux Dysphagia Autonomic dysfunction

21. Gait in cerebral palsy Idiopathic toe walking Spastic knee gait Crouch gait Gait

22. Determine grades of muscle strength and selective control. Evaluate muscle tone and determine type. Evaluate degree of deformity / contracture at each joint. Assess linear, angular and torsional deformities of spine, long bones, hands and feet. Appraise balance, equilibrium and standing / walking posture. Clinical Assessment Goals of Physical Examination

23. History Examination X-ray skull-intracranial calcification EEG CT/MRI Test of hearing ,vision IQ test Diagnosis

24. Achievable goals should be set The child with CP becomes the adult with CP Goals based on needs of adults Communication : verbal / nonverbal Mobility Walking Activity of daily living (ADL) feeding, dressing, toileting, bathing Turn focus of parents from the disease to the goal-oriented approach Goals of Management (Treatment)

25. Control of spasticity Physical therapy Orthotics Orthopedic surgery Types of Management (Treatment)

26. Spasticity is present in most patients with CP (65 % ) When it is reduced patients may : - perform integrated muscle movement - develop muscle strength - function at a higher level Approaches : Selective dorsal rhizotomy Intrathecal baclofen Botulinum-A toxin CONTROL OF SPASTICITY

27. Other oral medicines used in Cerebral Palsy: Dantrolene Flexeril Antiepileptic drugs such as Phenytoin , Sodium Valproate , Carbamazepine , etc.

28. Conventional PT : Works on muscles, tendons, and ligaments Active exercises Passive ROM exercises Passive stretching Bracing PHYSICAL THERAPY

29. Involve parents as much as possible (even if they resist) Do not raise false hopes which could increase frustration Contd…

30. ORTHOTICS Casting .. Short leg casts are applied with extended toe plates, careful molding of the heel and metatarsal head control. For a period of time varies but usually a minimum of 6 weeks. and is followed by the use of orthoses. There is a limited role for casting in patients with cerebral palsy. Orthoses .. Can be helpful in improving gait in ambulatory patient with cerebral palsy. Ankle-foot orthoses are most commonly prescribed to assist the child with positioning of the ankle and foot during gait.

31. Ankle Foot Orthoses (AFO)

32. m Orthoses

33. Primary deformity : needs treatment Compensatory deformity : can improve without intervention Surgery Prevent structural changes - usually early Improve function - usually later ORTHOPEDIC PROCEDURES Distinguish between

34. Type : spastic Extent : hemiplegics / diplegics : good results quadriplegics : minimal improvement Age : 3- 12 years IQ : good Good upper limb function : for walking Underlying muscle power : not weak Walker / non-walker : surgery hardly changes state but improves gait Prerequisites for effective surgery

35. For structural changes : Early e.g. Hip subluxation , usually <5 years To improve function ( gait ) : defer until walking ( independently / with aids ) until gait pattern develops and can be assessed walking : 18 – 21 months in hemiplegia 3 – 4 years in spastic diplegia Optimum time of lower extremity surgery 5 – 7 years: can analyze and observe gait pattern Timing For Orthopaedic Surgery

36. Timing For Orthopaedic Surgery Surgery should not be unduly staged one by one ( with each birthday ) ?ETA ? Hams ? Psoas ? Rectus Femoris Equinus Crouch Flexion Stiff Knee Ok

37. Hip Flexion contracture Increased hip addduction / scissoring Subluxating or dislocating hip Rotational deformities of femur and tibia Knee Foot Equinus Equinovarus Pes valgus Ankle valgus Hallux valgus Dorsal Bunion Lower limb involvement

38. Adduction and flexion deformity Hip at risk Hip subluxation Hip dislocation Hip Problems

39. Hip flexion contractures are found most commonly in patients with spastic diplegia and spastic quadriplegia. Flexion contracture is due to increased tone in the hip flexors (primarily the iliopsoas ) and relative weakness of the hip extensors (such as the gluteal muscles) The contracture is identified during the physical examination by performing the Thomas and Staheli maneuvers. Flexion Deformities in Hip

40. Thomas test Staheli test

41. Clinical Assessment Hip Flexors Ely / Rectus Femoris Test

42. Flexion internal rotation deformity should be differentiated from True Adduction deformity. Increased femoral anteversion when combined with crouch at the knee can produce the appearance of scissoring which termed as pseudo adduction Adductor surgery will be ineffective in improving the child’s ability to walk when the narrow base of gait is secondary to pseudo adduction.

43. Single-stage multilevel procedures are preferable to staged single-level procedures because hospitalization, immobilization, and rehabilitation time and the number of anesthetic exposures are decreased Hip flexion contractures from 15 to 30 degrees usually are treated with psoas lengthening Contractures of more than 30 degrees may require more extensive releases of the rectus femoris , sartorius , and tensor fasciae latae and the anterior fibers of the gluteus minimus and medius , in addition to the iliopsoas Treatment

44. Iliopsoas recession ( Skaggs et al technique ) Contd…

45. Iliopsoas release at the Lesser trochanter(Miller technique )

46. Iliopsoas tenotomy / lengthening / recession Tenotomy : Should not be done in ambulatory patients Recession : Doesn’t cause excessive hip flexion weakness Lengthening (z plasty) : best / easy /satisfactory in ambulating patients no risk of too much weakening of flexion power Contd…

47. Most common deformity Spasticity in the adductor muscles in cerebral palsy results in a narrow base of gait and scissoring, hip subluxation, and, in severely affected children, difficulty with perineal hygiene Adduction deformities in Hip

48. Adductor tenotomy and release Resection of tendon of adductor longus and anterior half of addductor brevis and gracilis ,if required Avoid neurectomy of ant. branch of obturator nerve Operative treatment:

49. Deformities of the hip in patients with cerebral palsy range from mild painless subluxation to complete dislocation with joint destruction, pain, and impaired mobility In most patients, the hip is normal at birth, and radiographic changes typically become apparent between 2 and 4 years of age. Subluxation and dislocation

50. Hip subluxation in patients with cerebral palsy can be difficult to detect clinically Routine clinical and radiographic examinations should be done every 6 months A practical radiographic method for quantifying the amount of hip subluxation present, which was described by Reimers as the “migration percentage.”

51. Hip subluxation ( partially out) Hilgenreiner line Contd… Dislocated Subluxated 50 % Reimer’s migration index

52. Hip subluxation ( partially out ) ( > 30 % uncoverage / broken Shenton’s line ) Contd…

53. Hip at risk Because early intervention can be very effective in preventing or delaying the development of dislocation, considerable work has been done to identify hips at risk

54. Hip at Risk Hip subluxation Contd… At 2.5 years At 7 years

55. Varus Derotational Osteotomy , usually combined with soft-tissue releases, is indicated for patients with excessive anteversion and valgus deformity of the proximal femur and a hip that is either subluxated or dislocated Operative Treatment

56. Combined One-Stage Correction of Spastic Dislocated Hip (San Diego Procedure) medial approach (soft-tissue release) anterior approach (open reduction) lateral approach (femoral osteotomy ) anterior approach ( pericapsular pelvic osteotomy ) Operative treatment

57. Proximal Femoral Resection Painful dislocated > 1 year Surgical resection

58. Hip Arthrodesis The ideal candidate is a patient with unilateral disease and no spinal involvement. Hip arthrodesis may be preferable in ambulatory patients because it allows weight bearing, in contrast to proximal femoral resections. Contd…

59. Deformities of the knee rarely occur in isolation The hip and the knee are tightly coupled because of the muscles that cross both joints, the “two-joint muscles.” KNEE

60. Flexion Deformity Recurvatum of the Knee Knee Valgus Patella Alta Deformities in knee

61. Most common knee deformity in patients with cerebral palsy and frequently occurs in ambulatory children Crouching gait. Flexion Deformity

62. Straight leg raising Angle< 70 … Indication The Hamstring Test Holt’s method Popliteal Angle< 135 - Indication Clinical Assessment Knee Flexion

63. Fractional Lengthening of Hamstring Tendons Operative management

64. Distal Transfer of Rectus Femoris

65. Combined Hamstring Lengthening and Quadriceps Shortening along with posterior capsule release

66. Hip flexion Deformity increases after hamstring release Better to transfer hamstring insertion to keep hip extended Effect of Knee Surgery on The Hip

67. Caused by Quadriceps spasticity or long standing Knee FFD. Can lead to repeated to micro trauma to patellar tendon and quadriceps tendon and stress fractures of patella and tibial tubercle. Usually painless, so intervention is not required. Correction of FFD of knee with hamstring lengthening causes improvement. Patella alta

68. DEFORMITIES Equinus Deformity Varus or Valgus Deformity Equinovarus Deformity Equinovalgus Deformity Calcaneus Deformity Cavus Deformity Forefoot Adduction Deformity Hallux Valgus Deformity Claw Toes FOOT

69. Most common foot deformity in patients with cerebral palsy It is an increased plantar flexion due to a plantar flexion contracture or dynamic plantar flexion due to over activity of the gastrocsoleus during gait Equinus deformity

70. Toe-walking patients must be considered as two different groups: equinus patients as a consequence of crouch at the hip and knee with natural ankle. Cerebral palsy must be differentiated from: Idiopathic toe walking as a congenital short Achilles tendon Muscular dystrophy (as Duchenne’s )produces toe walking.

71. Equinus.. Clinical examination.. Inability to fully dorsiflex the ankle The Silverskiold test: If the ankle can be passively dorsiflexed with the knee bent to 90 degree but cannot be dorsiflexed with the knee extended it is believed that the gastrocnemius is tight, but the soleus is not contracted This test is used to determine which type of surgical lengthening to perform

72. The Silfverskiold test

73. Dynamic : Treat by : Bracing Spasticity reduction Surgery Fixed : Treat by : Serial casting Surgery Treatment

74. Open Lengthening of the Achilles Tendon Surgical treatment

75. Z- Plasty Lengthening of the Achilles Tendon

76. Percutaneous Lengthening of the Achilles Tendon

77. Lengthening of the Gastrocnemius- Soleus Muscle Vulpius technique.. Strayer procedure

78. Baker technique (tongue-in-groove)

79. Diplegic patients typically have internally rotated and adducted hips, flexed knees, and external rotation deformity of the tibia. This combination of deformities causes the foot to assume a valgus position. In hemiplegic patients, the internally rotated thigh with the knee coming to full extension in stance phase causes the foot to internally rotate and produce a varus deformity Varus or Valgus Deformity

80. Muscle imbalance in which the invertors of the foot over power the evertors , most commonly Tibialis post. Gastrocnemius contributing equinus Surgery is indicated to Improve foot contact. Relieve pain. Relieve skin changes Equinovarus Deformity

81. Contd… The confusion test : The patient flexes the hip against resistance If supination of the forefoot is seen, then anterior tibialis is contributing to equinovarus deformity .When dorsiflexion is seen without supination , the deformity is less likely to respond to surgery on the anterior tibialis

82. It also is important to determine whether or not the deformity is flexible and correctable or rigid because patients with flexible deformities are more likely to be successfully treated nonoperatively with orthotics and shoe modifications and operatively with soft-tissue procedures such as tendon lengthenings , releases, or transfers (usually of the abnormally active muscle) Patients with rigid varus deformities generally require bone procedures, such as calcaneal osteotomy . Contd…

83. Lengthening of the Posterior Tibial Tendon Z-Plasty Lengthening of the Posterior Tibial Tendon Step-Cut Lengthening of the Posterior Tibial Tendon Intramuscular lengthening of the Posterior Tibial Tendon Surgical treatment

84. Split Tendon Transfers S plit posterior tibial tendon transfer It is one of the most common procedures for equinovarus deformity treatment. The posterior one-half of the posterior tibialis tendon is rerouted posterior to tibia and woven into the peroneus brevis tendon . Contd…

85. Kaufer split transfer of tibialis posterior tendon

86. Kaufer split transfer of tibialis posterior tendon

87. Split anterior tibial tendon transfer The lateral one-half of anterior tibialis is detached from its insertion. Passed beneath the extensor retinaculum . Inserted through a bone tunnel into the cuboid . Foot is positioned in 5-10 deg. of dorsiflexion . Known as the Rancho procedure when done in combination with posterior tibialis lengthening. Contd…

88. Split transfer of tibialis anterior tendon

89. Heel varus will respond to calcaneal osteotomy . If the deformity is severe, and with rigid component of mid foot supination … Triple arthrodesis should be performed. Even with bony procedures, muscle imbalance must be corrected. Bony surgery in Equinovarus

90. Dwyer closing wedge osteotomy of calcaneus for varus heel

91. Pes valgus Occurs in up to 25% of patients with cerebral palsy. and most common in older diplegic or quadriplegic patients. Can be caused by spastic peroneal muscles, weakness of the p.tibialis and a tight gastrocsoleus. Radiographs should be obtained in standing position for the foot and ankle. Conservative treatment should vigorously pursued.. shoe inserts and orthoses may be adequate to relive pain and avoiding surgery.

92. Surgical treatment of Pes valgus Bony surgery is the only predictable alternative for full and lasting correction. Surgical options are… The Grice extra- articular arthrodesis. Lateral column (calcaneal neck) lengthening. Calcaneal osteotomy. Triple arthrodesis.

93. Grice extra- articular arthrodesis Modifications to the original procedure: Fibular graft was changed to iliac crest graft. Using internal fixation to keep the position of the subtalar joint in combination with cancellous iliac crest graft.

94. Calcaneal osteotomy Advantage : preserve joint motion of sub talar joint. Results have been very good. Contraindications : severe rigid valgus deformity.

95. Triple Arthrodesis Treatment of choice for rigid symptomatic Pes valgus in adolescent with cerebral palsy. Resecting the subtalar , calcaneocuboid , talonavicular joints. Indications: Pain Skin ulceration over the talar head. Deformity interfering with ambulation in child with deformity not amenable to osteotomy . Satisfactory outcomes are found when deformity is well corrected. Degenerative changes have been documented in the ankle joint at an average of 18yrs.following arthrodesis in 43% of the pediatric population.

96. Triple arthrodesis for Pes valgus treatment

97. Dorsal bunion It is a rare deformity. The first metatarsal head is elevated, but the great toe is plantar flexed. Surgical rebalance depends on… Transfer of the flexor tendon to the extensor. Or flexor tenotomy . Or by transferring of the flexor hallucis brevis to the metatarsal neck Or by all that in combination with closing wedge plantar flexion osteotomy of first metatarsal.

98. Hallux valgus Develops in cerebral palsy patients in response to a Equino valgus deformity of the hind foot. There is a progressive eversion and abduction of foot because of peroneus longus is spastic. The toe is pushed laterally as weight is borne by the everted foot. The big toe comes to lie beneath the second toe, and the first metatarsal head becomes uncovered and painful bunion develops. When this deformity is mild, surgical treatment of Pes valgus will halt the progression of toe deformity. Bleck & Goldner described soft tissue realignment including: Release of the adductor hallucis t. and lateral capsulotomy of the first metatarsophalangeal joint. combined with first metatarsal and proximal phalangeal osteotomy. McKeveer technique: First metatarsophalangeal fusion. That led to better results with less recurrence than soft tissue realignment. Preferred position for fusion is 15-25 deg. Of dorsiflexion and slight valgus.

99. Hallux valgus in 14 yrs. Old girl with cerebral palsy. Treated with metatarsophalangeal fusion.

100. Intoeing Usually caused by femoral ante-version Internal tibial torsion adds to Intoeing Not related to spasticity of internal rotators If severe : Derotation osteotomy Delay to late childhood if possible Derotation osteotomy of femur might cause tightening of medial hamstrings ( might need lengthening )

101. In-toeing Normal anteversion Excessive anteversion Femoral anteversion

102. Why Does Anteversion Cause In-toeing ? Anteversion rotates Greater Trochanter posteriorly On stance phase need Abductor power Abductor power optimized when Greater Trochanter is lateral not posterior Internal rotation in stance phase brings Greater Trochanter laterally

103. In-toeing Indication for derotation osteotomy Severe femoral anteversion, with loss of all external rotation. Dynamic in-toeing causing gait abnormalities. Dynamic in-toeing causing secondary foot deformity. Usually not before 8-10 years.

104. THANK YOU …!!