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CEREBRAL PALSY Prepared by: Supervised by:

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1 CEREBRAL PALSY Prepared by: Supervised by:
Dr. Abdullah K. Ghafour Dr. Hamid Ahmed Jaff 2nd year IBFMS trainee

2 Introduction The term ‘cerebral palsy’ includes a group of disorders that result from permanent non-progressive brain damage during early development and are characterized by abnormalities of movement and posture. CP was first described by William Little in 1862. CP is the result of a brain lesion; therefore, the spinal cord and muscles are structurally and biochemically normal. William John Little He is an English surgeon Suffered childhood poliomyelitis with residual left lower extremity paraparesis, complicated by severe talipes He 1st identified Cerebral Palsy in his publication "On the Deformities of the Human Frame" in the 1860s One of the first to bridge the gap between neurology and orthopaedics CP was then known as "Cerebral Paralysis" or “Little’s Disease

3 Epidemiology Incidence of 1 to 3 per 1,000 live births.
the highest rates in premature babies and those of multiple births. 78 per 1,000 infants weighing less than 1,000 g. 9 to 12 per 1000 in twins 31 to 45 per 1000 in triplets 111 per 1000 in quadruplets.

4 Etiology Known causal factors: maternal toxaemia, prematurity, perinatal anoxia, kernicterus and postnatal brain infections or injury like birth injury. CP can affect childhood motor development, speech, cognition, and sensation. The main consequence is the development of neuromuscular incoordination, dystonia, weakness and spasticity

5 Classification Cerebral palsy is usually classified according to the type of motor disorder, with subdivisions referring to the topographical distribution of the clinical signs. TYPE OF MOTOR DISORDER: Spasticity Hypotonia Athetosis Dystonia Ataxia Mixed palsy

6 Classification Spasticity is the commonest muscle movement disorder and is associated with damage to the pyramidal system in the CNS. It is characterized by increased muscle tone and hyper-reflexia with passive stretch . “clasped knife”. Hypotonia Infants are described as floppy.it is usually a phase, lasting several years during early childhood before the features of spasticity become obvious. CP affectd

7 Classification Athetosis manifests as continuous, involuntary, purposeless movements that may be exacerbated by environmental stimulation. It is caused by damage to the extrapyramidal systems of the CNS. In pure athetoid cerebral palsy, joint contractures are unusual and muscle tone is not increased. Dystonia There is a more generalized increase in muscle tone and abnormal positions induced by activity “lead pipe”. This occurs in the absence of hyperreflexia, spasticity, and clonus and it may occur with athetosis.

8 Classification Ataxia appears in the form of muscular incoordination during voluntary movements. It is usually due to cerebellar damage. Balance is poor and the patient walks with a characteristic wide-based gait. Mixed palsy appears as a combination of spasticity and athetosis. The presence of both types of motor disorder can make the results of surgical intervention unpredictable.

9 TOPOGRAPHIC DISTRIBUTION
Hemiplegia Diplegia Total body involvement (Quadriplegia)!! Monoplegia

10 TOPOGRAPHIC DISTRIBUTION
Hemiplegia: is the commonest. This usually appears as a spastic palsy on one side of the body with the upper limb more severely affected than the lower. Most of these children can walk and they respond reasonably well to treatment. Diplegia: involves both sides of the body, with the lower limbs always most severely affected. Side to side involvement may be asymmetrical, Intelligence is often normal.

11 TOPOGRAPHIC DISTRIBUTION
Total body involvement: general and often more severe disorder affecting all four limbs, the trunk, neck and face with varying degrees of severity. Patients usually have a low IQ, they may have epilepsy, they are often unable to walk and the response to treatment is poor. Monoplegia: very rare, occasionally appears in an upper limb.

12 gross motor function classification system (GMFCS)
GMFCS is most commonly used to describe the patient’s level of function before and after an intervention. The GMFCS scale which was developed by Palisano et al. in 1997 , has five levels: GMFCS 1: ambulates without aids on all surfaces and keeps up with peers. GMFCS 2: fully ambulatory, may use lower extremity orthoses, and does not keep up fully with peers. GMFCS 3: uses ambulatory aids such as a walker or crutches and may use a wheelchair for longer distances. GMFCS 4: nonambulatory patients who are able to propel their own wheelchair.

13 gross motor function classification system (GMFCS)
GMFCS 5 : inability to transfer, propel a wheelchair, or support the trunk. GMFCS levels: level 1 35% level 2 16.4%; level 3 14.2% level 4 16.1% level 5 18%

14 Diagnosis Diagnosis in infancy:
The full-blown clinical picture may take months or even years to develop. history of prenatal toxaemia, haemorrhage, premature birth, difficult labour, foetal distress or kernicterus should arouse suspicion. A neonatal ultrasound scan of the head may identify intracerebral bleeding that would increase the likelihood of later problems. Early symptoms include difficulty in sucking and swallowing, with dribbling at the mouth. Motor milestones are delayed.

15 Diagnosis Diagnosis in later childhood:
Most children have already had the diagnosis made. the child should be carefully observed sitting, standing, walking and lying. gross motor function classification system (GMFCS) is used to evaluate there condition, which categorizes the child, relative to their age, in terms of mobility and bases this on their average function There are no definitive laboratory studies

16 Diagnosis GAIT assessment: Gait should be observed with and without shoes or orthotic supports. Clinical gait analysis; Each limb must be observed in both the stance and swing phases of gait and in the coronal, sagittal and transverse planes. Computerized gait analysis; it supplements clinical gait analysis, includes Kinematics, kinetics, EMG, pedobarography (foot pressures), and metabolic energy analysis. Sutherland and Davids at (1993) described four pathological gait patterns based on knee motion in the sagittal plane: Toe-walking (jump knee) gait crouch knee gait Stiff knee gait Recurvatum knee gait Toe-walking, recurvatum, crouch and stiff knee

17 Diagnosis Toe-walking (jump knee) gait: due to tightness in the hamstring muscles, hip adductors and gastronomies muscles crouch knee gait; due to long lasting of the knee extensor muscles mainly rectus femoris in stance phase .this also causes pelvis to bend forward and causes a crouch in body. Stiff knee gait; due to stiffness in the posterior capsule of knee in the flexed position Recurvatum knee gait; due to long lasting of the knee flexor muscles mainly biceps femoris in stance phase . A B C D

18 Diagnosis Imaging studies: Deformity assessment:
MUSCLE CONTRACTURE: longstanding spasticity leads to relative shortening of the muscles and hence fixed contractures and changes in joint congruity. BONY DEFORMITY: it is influenced by muscle contracture. STRUCTURAL SCOLIOSIS: Flexible curves are common in CP, but it may become structural. Imaging studies: CT & MRI of the brain Electroencephalography: Important in the diagnosis of seizure disorders Electromyography and nerve conduction studies: Helpful when a muscle or nerve disorder is suspected

19 management There is no single ‘blueprint’ for the management of all patients with cerebral palsy; each patient and his or her family provides a different challenge. For all patients with cerebral palsy the priorities are: an ability to communicate with others an ability to cope with the activities of daily living (including personal hygiene) independent mobility , which may mean a motorized wheelchair rather than walking.

20 management For the child who from an early age is recognized to be ‘non-walking’ realistic goals should be: a straight spine with a level pelvis located, mobile and painless hips that flex to 90 degrees (for comfortable sitting) and extend sufficiently to allow comfortable sleeping and participation in standing/swivel transfers knees that are mobile enough for sitting, sleeping and transferring plantigrade feet that fit into shoes and rest on the footplates of the wheelchair comfortably.

21 management TONE MANAGEMENT: Medical treatment:
anticonvulsants for seizures, short-term benzodiazepine use for postoperative pain trihexyphenidyl for dystonia. oral tizanidine (muscle relaxant ) has been used to relieve spasticity Physical therapy; is considered to be most helpful in early childhood up to the age of 7 or 8 years Positioning and splinting; Splints are used to prevent muscle contracture, maintain joint position and improve movement and hence function. Manipulation and serial casting: have limited role.

22 management Intrathecal Baclofen:
γ-aminobutyric acid agonist, acts at the spinal cord level to impede release of the excitatory neurotransmitters that cause spasticity. implantable pumps filled with baclofen are surgically inserted into the anterior abdominal wall and the dose of medication is titrated. Continuous infusion of the medication is delivered by the pump, which requires refilling approximately every 3 months. Studies have shown decreased upper and lower extremity tone and improvements in range of motion.

23 management Botulinum Toxin:
blocks the release of acetylcholine at the neuromuscular junction. botulinum toxin injection is of use in the treatment of lower extremity spasticity. It is injected at known anatomic sites of innervation, often guided by EMG. The drug begins taking effect after 2 to 3 days, and its effect wears off after approximately 3-6 months. botulinum toxin may allow tendon surgery to be delayed until a later age, when the risk for recurrence is lower.

24 management Selective dorsal rhizotomy;
Neurosurgical procedure in which selected dorsal nerve roots from L1 to S2 are severed at the level of the cauda equina to reduce spasticity. Candidates must have purely spastic CP. Studies have shown decreased tone and increased joint range of motion after rhizotomy Residual contractures, hip subluxation, and spinal deformity, in general, may occur in 36% of selective dorsal rhizotomy patients.

25 management Operative treatment: The indications for surgery are:
a spastic deformity which cannot be controlled by conservative measures fixed deformity that interferes with function secondary complications such as bony deformities, dislocation of the hip and joint instability. Shoe wear problems Pain Perineal hygiene problems

26 Timing of Surgery in CP Delaying surgery is advocated until age of 7–8 years because development of the CNS and the gait pattern matures around this age and then doing all the necessary operations at one or two sittings. some patients surgery cannot be delayed like hip subluxation which should undergo surgery when the problem is first recognized to improve coverage of the hip. children who are nearly ambulatory but whose progress has been halted by contractures, earlier surgery may allow them additional range of motion to make walking less cumbersome.

27 The Role of Orthopedic Surgery in the Management of CP
Management of Foot Involvement: Equinus deformity: selective lengthening of the Achilles tendon Lengthening may be performed by open or percutaneous techniques. The tendon must be repaired with sufficient tension to avoid postoperative calcaneus gait ankle is then immobilized in a short-leg cast for 6 weeks gastrocnemius fascial recession the aponeurosis of the gastrocsoleus is divided in chevron fashion the soleus muscle fibers are not disturbed Immobilization is minimized FIGURE Lengthening of the gastrocnemius by the Vulpius technique.

28 The Role of Orthopedic Surgery in the Management of CP
Management of Foot Involvement: Equinovarus deformity: Posterior Tibialis Tendon Lengthening: usually done in conjunction with an Achilles tendon–lengthening patient is placed in a short-leg cast for approximately 6 weeks. Transfer of the Posterior Tibialis Tendon to the Dorsum: Calcaneovalgus can be a disastrous result and occurs in up to 68% of patients. The only indication is when the posterior tibialis is Completely silent during stance phase and active during swing phase.

29 The Role of Orthopedic Surgery in the Management of CP
Management of Foot Involvement: Equinovarus deformity: Split Posterior Tibialis Tendon Transfer: posterior half of the posterior tibialis tendon is detached from its insertion, split proximally to a level just proximal to the ankle, rerouted posterior to the tibia and fibula, and then re- attached into the tendon of the peroneus brevis.

30 The Role of Orthopedic Surgery in the Management of CP
Management of Foot Involvement: Equinovarus deformity: Split Anterior Tibialis Tendon Transfer: the lateral half of the anterior tibialis is detached from the base of the first metatarsal. The tendon is passed beneath the extensor retinaculum and inserted through a bone tunnel into the cuboid bone. usually combined with an Achilles tendon–lengthening procedure. Bone Surgery: If the varus deformity of the foot is fixed. heel varus will respond to calcaneal osteotomy. If the deformity is very severe, calcaneal osteotomy will be insufficient and triple arthrodesis should be performed.

31 The Role of Orthopedic Surgery in the Management of CP
Management of Foot Involvement: Pes Valgus deformity: Valgus deformity of the foot occurs in up to 25% of patients with CP Conservative treatment should be vigorously pursued because shoe inserts and orthosis modifications may be adequate to relieve the pain In valgus deformity, bone surgery is the only predictable alternative for full and lasting correction. Surgical options are: the Grice extraarticular arthrodesis ( extraarticular fusion of subtalar joint with bone graft) Lateral column lengthening of the calcaneal neck Calcaneal osteotomy triple arthrodesis.

32 The Role of Orthopedic Surgery in the Management of CP
Management of Foot Involvement: Ankle valgus: Surgical correction of ankle valgus is performed by either: Hemiepiphysiodesis of the distal medial aspect of the tibia provides gradual correction When immediate correction is desired, distal tibial osteotomy is useful. Hallux Valgus develops in response to an equinovalgus deformity of the hindfoot first MTP arthrodesis is preferred for the surgical treatment of hallux valgus in patients with CP

33 The Role of Orthopedic Surgery in the Management of CP
Management of knee Involvement: Hamstring Lengthening: The hamstrings are nearly always affected in patients with CP. hamstring spasticity can be measured via the popliteal angle. Normal popliteal angles are variable, with a mean value of 26 degrees in children 4 years and older. Values greater tha 50 degrees are considered abnormal. 1) Orthotic Management: Mild tightness in the hamstrings may respond to orthotic management, usually with ground reaction AFOs. 2) Surgical lengthening of the distal hamstrings is the preferred surgical treatment of crouched-knee gait

34 The Role of Orthopedic Surgery in the Management of CP
Management of knee Involvement Rectus Femoris Transfer: Surgical treatment of stiff-knee gait and inability to flex the knee in swing phase consists of rectus femoris transfer. It is often performed simultaneously with hamstring lengthening The tendon is divided transversely just proximal to the superior pole of the patella, then passed medially and sewn into the stump of the gracilis tendon Bone surgery: Bone surgery has become accepted for patients with CP who have fixed flexion contractures or those who have previously undergone hamstring lengthening. distal femoral extension osteotomy +/- patellar tendon reefing is useful in older children and teens.

35 The Role of Orthopedic Surgery in the Management of CP
Management of Rotational Abnormalities of the Femur and Tibia Spasticity in the lower extremities over time leads to the development of rotational abnormalities in the femur and tibia (increased femoral anteversion and external tibial torsion ). Derotational Osteotomy : Femoral anteversion is treated by femoral osteotomy, either proximally at the intertrochanteric or subtrochanteric level or distally at the supracondylar level. In tibial rotational deformities, surgical correction should be performed at the distal level. Proximal osteotomies are associated with a higher risk for neurovascular injury.

36 The Role of Orthopedic Surgery in the Management of CP
Management of Hip Involvement in CP: Hip Flexion Contracture: Hip flexion contractures are nearly always seen in combination with increased hip adduction and internal rotation, with knee flexion secondary to hamstring spasticity, and equinus, or valgus deformities of the feet. Hence, surgery to improve hip flexion contractures is part of the SEMLS( Single Event Multilevel Surgery) approach in conjunction with other soft tissue or bony procedures in patients with CP. It is caused by increased tone in the hip flexors, primarily the iliopsoas muscle. The contracture is identified by performing the Thomas and Staheli maneuvers The recommended procedure is psoas tenotomy performed over the pelvic brim.

37 The Role of Orthopedic Surgery in the Management of CP
Management of Hip Involvement in CP: Adduction Contracture: Spasticity in the adductor muscles in CP results in a narrow base of gait and scissoring. The muscles leading to the adduction contracture are the adductor longus, adductor brevis, adductor magnus, gracilis, and occasionally the pectineus. Surgery to improve adduction contractures is limited to adductor release, with or without obturator neurectomy, and posterior adductor transfer.

38 The Role of Orthopedic Surgery in the Management of CP
Management of Hip Involvement in CP: Hip Subluxation or Dislocation: Hip dysplasia or instability is a common problem in patients with CP and occurred in approximately 21% Because bony deformity develops in response to muscular spasticity, bone surgery in the absence of soft tissue release is ineffective in correcting subluxation or dislocation secondary to CP. The goals of treatment include a painless hip that allows stable sitting and positioning in a non-ambulatory patient and full hip reduction in an ambulatory patient. Categories of Surgical Treatment. divided into three categories (1) soft tissue release for subluxation or a hip at risk (2) reduction and reconstruction of the subluxated or dislocated hip (3) salvage surgery for long-standing painful dislocations.

39 The Role of Orthopedic Surgery in the Management of CP
Management of Hip Involvement in CP: Hip Subluxation or Dislocation: First category: hip at risk is defined as a hip that has significant adduction and flexion contractures but minimal subluxation, Surgical treatment is aimed at preventing dislocation ofthe hip. The procedure consists of adductor release and iliopsoas lengthening or release. Second category: The most frequently performed procedure in this setting is femoral varus derotation osteotomy (VDRO). Third category: Indications for surgery in this group of patients include hip pain, inability to sit in a modified wheelchair, and difficulty with perineal hygiene. Four surgical options are available: proximal femoral resection, valgus osteotomy of the proximal end of the femur, hip arthrodesis, and total hip arthroplasty.

40 The Role of Orthopedic Surgery in the Management of CP
Management of Hip Involvement in CP: Hip Subluxation or Dislocation: varus derotation osteotomy proximal femoral resections Valgus osteotomy

41 The Role of Orthopedic Surgery in the Management of CP
Management of Upper Limb Involvement in CP: Operative treatment of the upper limb can improve the function of children with CP. Basic goals are improvement in function and improvement in appearance upper limb deformities: (1) Elbow flexion deformity: no treatment is needed, occasionally fractional lengthening of the biceps and brachialis tendons with release of the brachialis origin is done. (2) Forearm pronation deformity: fairly common, release of pronator teres may improve the position. (3) Wrist flexion deformity: it is usually in an ulnar direction it can be improved by lengthening or releasing flexor carpi ulnaris. In severe cases wrist arthrodesis is done.

42 The Role of Orthopedic Surgery in the Management of CP
Management of Upper Limb Involvement in CP: (4) Flexion deformity of the fingers: The flexor tendons can be lengthened individually, but if the deformity is severe a forearm muscle slide may be more appropriate. (5) Thumb-in-palm deformity: due to spasticity of the thumb adductors and/or flexors. but late there is also contracture of flexor pollicis longus. In mild cases, function can be improved by splinting. Resistant deformity may need combined lengthening of flexor pollicis longus and release of the thenar muscles.

43 The Role of Orthopedic Surgery in the Management of CP
Management of Spinal Deformity in CP: Scoliosis: Scoliosis is a significant problem in children with CP that affects between 25% and 68% of patients. Scoliosis leads to difficulty sitting, the functional position needed by a wheelchair-bound child. Nonoperative treatment of scoliosis with adaptive seating and orthoses has not met with success. Indications for spinal fusion are: 1. Curves greater than 40 degrees in ambulatory patients 2. Progressive curves greater than 50 degrees in communicative patients 3. Curves that interfere with seating and nursing in patients whose families desire surgical correction

44 The Role of Orthopedic Surgery in the Management of CP
Management of Spinal Deformity in CP: Spondylolysis and Spondylolisthesis: hip flexion contractures and increased lumbar lordosis may lead to an increased incidence of spondylolysis and back pain in patients with CP which account for in 21% of patients. Dorsal rhizotomy may predispose patients to spondylolisthesis inasmuch as 12% of patients Treatment is surgical.

45

46 references Solomon L., Warwick D. , Nayagam S.,[2010] Apley’s System of Orthopaedics and Fractures, 9th ed. Hodderarnold comp.,London, UK. Miller M. , Thompson S. , Hart J. ,[2012] REVIEW OF ORTHOPAEDICS [PDF], 6th ed. by Saunders, an imprint of Elsevier Inc. , Philadelphia, USA. Canale S. , Beaty J. , [2007] Campbell’s Operative Orthopaedics [PDF], 11th ed. By Mosby, An Imprint of Elsevier , Tennessee, USA. John A. Herring, [2014] Tachdjian’s Pediatric Orthopaedics [PDF], 5th ed. . by Saunders, an imprint of Elsevier Inc. , Philadelphia, USA. John M. Flynn, [2011] American Academy of Orthopaedic Surgeons, Orthopaedic Knowledge Update OKU 10 [PDF] , Printed in the USA. ORTHOPAEDIC REVIEW [2015] by orthobullets, [PDF], Collected By Islam Gomaa Beltage.

47 "My hand trembles, my heart does not."
Christy Brown RJ Mitte Stephen Hopkins; "My hand trembles, my heart does not." Jhamak Ghimire Maysoon Zayid


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