Understanding Cerebral Palsy: Causes and Treatments
Understanding Cerebral Palsy: Causes and Treatments
Cerebral Palsy
Was first described by William Little in 1862. Then it was known as Little disease. The term Cerebral palsy originated with Freud.
Definition
In all cases the following must be true Cerebral palsy is the result of a brain lesion. Therefore, the spinal cord and muscles are structurally and biochemically normal. The brain lesion must be fixed and non progressive. Thus, all of the progressive neuro degenerative disorders are excluded from the definition. The abnormality of the brain result in motor impairment.
Epidemiology
The Incidence is between 2.4-2.7 per 1000 live births. The risk of cerebral palsy in a child born full term is app.1 in 2000. The incidence has been correlated with gestational age and birth weight.
Etiology
Divided into 3 time periods Prenatal Maternal infection and toxins (TORCHES). Fetal exposure to drugs and alcohol through maternal .(cocaine, heroin ,marijuana) Congenital malformations of the brain that occur during early pregnancy. Rhesus blood group incompatibility resulting in kernicterus. Maternal health problems. ex: RF-Infections. Prenatal chorioamnionitis and maternal infections and placental abnormalities. Perinatal Anoxia as a result of perinatal complications. Fetal distress. Premature delivery. Sepsis in neonatal period. Bronchopulmonary dysplasia and prolonged ventilation in preterm infants. Heart surgery before the age of 1mnth. Postnatal Infections in early childhood (meningitis). Any episode of hypoxia. Trauma and head injuries.
Classification
Physiologic classification Describes the type of movement disorder present Spasticity: the most common. Increased tone in the extremities.. (the Clasped knife model) Hypotonia: usually a phase, leading most frequently to spasticity. Dystonia: lead pipe model. Athetosis: abnormal writing movements, patient cannot control. Ataxic: in cerebellar lesions. Patients frequently have a mixed form of movement disorders. Geographic classification Describes what part of body is affected.. Hemiplegia: one side, upper usually more involved. Diplegia: both sides, with both lower extremities and lesser involvement of upper extremities. Triplegia: both lower and one upper extremity. Quadriplegia: Total body involvement.
Spastic Diplegia
Spastic quadriplegia
Evaluation
Diagnosing.. Obtaining a complete history (birth history, birth weight, complications following birth..) Asking about the childs preferential use of one hand or leg. Related medical conditions (seizures, speech disorders) Physical examination.. Increased muscle tone. Deep tendon reflexes are increased. Fine motor activities testing. Retained infantile reflexes. Balance, sitting and gait of child.
Treatment
Treatment, Surgical or nonsurgical, must be goal oriented.. The goals of treatment that have linked to productive lives as adults are: Communication, education, mobility and ambulation. Treatment ranges from observation, physical therapy, medications.. to surgery.
Treatment..
Physical therapy.. Often the first rendered to the child with cerebral palsy. No controlled studies have confirmed that regular physical therapy improves the out come of the child with cerebral palsy. The approach to physical therapy is to establish a therapy to monitor the developmental milestones of the very young child around the age 2-3 years. Therapy continue if gains are being made in attaining ambulation.
Treatment
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.
Cerebral palsy
The major surgical methods Lengthening of a tight musclotendinous unit. Augmenting a weak muscle by tendon transfer. Arthrodesis in the older children.
Surgical treatment.. Most procedures attempt to restore balance by combination of soft tissue releases and tendon transfer, or by arthrodesis where soft tissue procedures are inadequate.
The typical posture of spastic upper limb iselbow, wrist and fingers flexion, and forearm pronation.
Occurs usually in the globally involved child. Shoulder adduction contracture may be present Severe elbow flexion contracture can be relieved by
i. ii. iii. iv.
Release of the lacertus fibrosis. Z-lengthening of biceps tendon. Lengthening of brachialis tendon. Proximal origin of brachoradialis m. can be released.
Forearm pronation
Develops insidiously, and with growth, causes a rotational deformity of radius. The release or rerouting of the pronatore tendon is the option for correction when the fore arm is passively correctable.
The goal of surgical procedure on the wrist and fingers is to allow the fingers to open with wrist flexion for release, and to close with wrist extension for grasp. The lengthening of the wrist flexors, flexor carpi ulnaris at the intramuscular level, Z- lengthening of flexor carpi radialis, release of palmaris longus and super fascia and with fractional lengthening of the flexor profundus. all that will allow the wrist to be brought into extended position. The extensor carpi ulnaris is the preferred tendon to transfer into the extensor carpi radialis brevis.
Severe deformity: especially in non functional hand, wrist arthrodesis is a solution for hygiene and care problems.
The thumb
Thumb-in-palm deformity is common in cerebral palsy patients. The deforming forces are: adductor pollicis and all the intrinsic muscles. The approach is to release contracted soft tissue and then augment weak extensors and abductors by 1. Simple release of the contracture when child demonstrates active thumb interphalangeal extension and a palpable extensor pollicis longus. 2. Release of the origins of the thenar musculature, and the two heads of adductor pollicis. 3. Release of the first dorsal interosseous fascia with release of the thumb-index web space.
Cerebral palsy
Foot surgery
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. Toe-walking patients must be considered as two different groups: i. equinus patients. ii. as a consequence of crouch at the hip and knee with natural ankle. Cerebral palsy must be differentiated from: A. Idiopathic toe walking as a congenital short Achilles tendon B. Muscular dystrophy (as Duchenns)produces toe walking.
Equinus..
Clinical examination.. Of the child with equinus due to cerebral palsy shows inability to fully dorsiflex the ankle.
The Silverskiold test: If the ankle can be passively dorsiflexed with the knee bent to 90 deg. but cannot be dorsiflexed with the knee extended.. Its felt that the gastrocnemius is tight, but the soleus is not contracted.
Equinus
Leads to Gait is less efficient. Foot drop results if anterior tibialis is unable to lift the foot to natural during swing phase. Genu recurvatum is seen. Valgus positioning of the hind foot. Calcaneus is actually in equinus. Pain and callosities result over the head of talus. Hallux valgus can develop.
1. 2.
Gastrocnemius recession..
1.
2.
3.
3.
4.
5.
Baker technique
Open technique.. with Z-fashion lengthening Per cutaneous techniques.. White.. Two-cut technique. Hoke.. Three-cut technique.
Muscle imbalance in which the invertors of the foot over power the evertors. with the gastrocnemius contributes equinus. Surgery is indicated to.. 1. Improve foot contact. 2. Relieve pain. 3. Relieve skin changes. Tendon surgery can be done if the foot can passively corrected with manipulation to the natural position. Bony surgery is necessary when the deformity is stiff and cannot be manipulated into a plantigrade position foot.
Equinovarus deformity..
The confusion test: The patient flexes the hip against resistance..
If the supination of the forefoot is seen, the a.tibialis is contributing to equinovarus deformity.. When dorsiflex is seen without supination, the deformity is less likely to respond to surgery on the a.tibialis.
Equinovarus surgery
p.tibialis tendon lengthening
I. II.
Usually done in conjunction with Achilles tendon lengthening. It is done In young patients with mild varus with equinus. Can be performed as intramuscular lengthening or as tendon Z lengthening. Complications..
Recurrence of the deformity. Development of postoperative valgus.
Equinovarus surgery
Transfer of the p.tibialis tendon to the dorsum
It is not a preferred procedure because of its disastrous complications: heel valgus in 68% of patients.
Equinovarus surgery
Split p.tibialis tendon transfer
Popularized by Kaufer& Green. It is one of the most common procedures for equinovarus deformity treatment. The posterior one-half of the p.tibialis tendon is rerouted posterior to tibia and woven into the peroneus brevis tendon.
Equinovarus surgery
Split a.tibialis tendon transfer
The lateral one-half of a.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 p.tibialis lengthening.
Equinovarus surgery
Bony surgery
Its done where the varus deformity is fixed.. and the p.tibialis tendon lengthening would not provide correction. 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.
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.
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.
First described by Grice in 1952 for correcting Pes valgus in young polio patients in the age of 4-12 yrs. Then widely used for correction of Pes valgus in children with cerebral palsy. Satisfactory results reported in 79% of patients. A graft is propped into the sinus tarsi laterally to support the plantar flexed talus and correcting valgus of subtalar joint. Advantage: does not interfere with the growth of tarsal bones. Failures are due to:
1. 2. 3. 4. 5.
Persistent valgus. Overcorrection into varus. Ankle valgus. Graft slippage. Graft nonunion.
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.
Correction is achieved by lengthening the calcaneus, and therefore the lateral column of foot, effecting tightening of plantar fascia and reduction of the lapsed talonavicular joint.
Achilles tendon and peroneal tendons must be lengthened.
Treatment of choice for rigid symptomatic Pes valgus in adolescent with cerebral palsy. Resecting the subtalar, calcaneocuboid, talonavicular joints. Indications: 1. Pain 2. Skin ulceration over the talar head. 3. 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.
Ankle valgus
Valgus alignment of the ankle develops in patients with neuromuscular diseases. Radiographs of ankle in standing should be obtained before surgical correction of Pes valgus. Surgical correction is done by.. Hemiepiphysiodesis of the distal medial tibia (medial screw, staples or open epiphysiodesis) Osteotomy Osteotomy is useful when immediate correction is desired. A Closing wedge osteotomy of distal tibia, combined with distal fibular osteotomy is performed.
I. II.
Ankle valgus
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.
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.
Hallux valgus in 14 yrs. Old girl with cerebral palsy. Treated with metatarsophalangeal fusion.
Knee surgery
Knee surgery
Consists of Hamstring lengthening. Rectus femoris transfer.
Hamstring lengthening
The hamstrings are nearly always affected in cerebral palsy patients, and tight hamstrings lead to crouch gait. Hamstrings cross two joints, at the hip they serve as extensors, and at the knee they serve as flexors. The popliteal angle measures the hamstrings spasticity and values greater than 50 degrees are considered abnormal in children of 4yrs and older. (normal is about 26 degs.) Fixed knee flexion contracture develops in severe hamstring contracture. That leads to disappointing results following hamstring lengthening.
The Eggers procedure has been historically used for the correction of spasticity in the hamstrings and crouch knee, but genu recurvatum was a frequent complication.
Hamstring lengthening..
Surgical lengthening of the distal hamstring is now the preferred surgical treatment for crouch knee gait and is often performed in combination with other soft tissue procedures.
With intramuscular aponeurotic lengthening of semimembranous, a Z-lengthening of semitendonosus and either tenotomy or Z-lengthening of the gracilis at a level just proximal to the knee.
An adequate lengthening has been accomplished (intraoperatively) when the popliteal angle is reduced to around 20 degs. Greatest improvements in the knee flexor contracture are seen within 1 year following surgery.
Significant crouch gait during stance phase with limited knee extension at midstance. Increased popliteal angle and positive rectus femoris grab on clinical examination. If EMG shows activity in the rectus femoris during swing phase. In the case of sufficient hip pull-off power generation at late stance phase. For velocity greater than 60% of normal. If there are no significant rotational abnormalities of the hip that interfere with gait.
Rotational osteotomies
Spasticity in the lower extremities leads overtime to the development of rotational abnormalities in the femur and tibia. Persistent femoral anteversion is present in patients with spastic diplegia, and in some patients with severe spastic hemiplegia. Physical examination shows increased internal rotation and decreased external rotation of the hip. Overtime, compensatory external rotation of the tibia develops. Internal tibial torsion may be present in children with cerebral palsy, specifically patients with spastic hemiplegia.
Rotational osteotomies..
The best correction of rotational malalignment of the lower extremity is achieved by derotational osteotomies. Femoral anteversion is treated with inter or subtrochanteric level osteotomy or at the supracondylar level. In the case of tibial rotational deformities, surgical correction should be performed at the distal level.
Hip surgery
Hip surgery
Hip surgery in cerebral palsy can be divided into three areas: Correction of hip flexion contracture. Correction of increased hip adduction during gait or scissoring. Surgery to treat the subluxating or dislocating hip.
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. The sacro-femoral angle is used to objectively quantify of the hip flexion contracture. Normally should be between 45-65 degs.
Thomas test
Staheli test
hip dysplasia or instability is a common problem occurring in about 21% of cerebral palsy patients. Reports shows a prevalence of subluxation or dislocation ranging from 3 to 47% in cerebral palsy patients. Patients with spastic hemiplegia rarely develop hip dysplasia and dislocation. Patients with spastic diplegia are at increased risk. Patients with spastic quadriplegia have the highest rate of hip instability. The mean age at which patients with cerebral palsy present with subluxation or dislocation is 7 yrs. Hip subluxation develops in response to muscle imbalance. Subluxation develops gradually, with increasing lateralization and proximal migration of the femoral head with respect to the acetabulum.
Bony deformity occurs in response to the spasticity. The normal remodeling of the femoral anteversion seen in normal young child does not occur in patients with cerebral palsy, and anteversion persists into adulthood. The neck-shaft angle becomes increased as coxa valga develops. Acetabular changes occur as the hip subluxates,with an increased Acetabular angle and erosion of the lateral lip of acetabulum Finally, changes in the shape of femoral head takes place, with superolateral and superomedial notching.
Adduction contractures in a young child, no older than 5yrs. With limited abduction of 30 degs. or less. And a migration index of not more than 30%
Femoral osteotomy
The most frequently performed procedure in the treatment of more significant hip subluxation is a femoral varus derotation osteotomy (VDRO). An osteotomy is performed at the intertrochanteric level, usually accompanied by a closing wedge taken medially and/or femoral shortening, and the femur is fixed in an increased amount of varus with internal fixation. The two most frequently used plants for internal fixation are.. Hip screw and side plate. 90 degs. blade plate.
Femoral osteotomy..
Release of soft tissue contracture must also be done to balance the forces across the hip. Hoffer concluded that VDRO is a good procedure for hip subluxation, but is inadequate to treat the dislocated hip in cerebral palsy.. Other studies advocate pelvic osteotomy in addition to VDRO if the preoperative migration index is greater than 70% No significant remodeling into valgus can be expected in patients operated on the age 8yrs or older. Complications for femoral VDRO
Recurrent dysplasia. Loss of fixation. Fractures. Delayed union and nonunion are rare. Heterotopic ossification can result.
Presence of severe subluxation or, The presence of moderate incongruity on arthrography following the femoral osteotomy.
Paralytic dislocation of R hip in cerebral palsy+ open reduction +femoral shortening +Chiari osteotomy
This procedure is indicated in a hip in which spherical congruency cannot be achieved. and also helpful in hips in which there is global acetabular deficiency and a small articular surface. Studies of the results in cerebral palsy are encouraging, with hip stability obtained in 83-95% of patients. This procedure should not be performed in very young patients.
The osteotomy extends through the outer table of the ilium from the anterior iliac spine to the sciatic notch. A bicortical osteotomy is performed only at the anterior-inferior iliac spine, and the inner table of ilium is not cut. The lateral osteotomy is extended to the triradiate cartilage under fluoroscopic guidance. The osteotomy is pried down laterally and posteriorly. Wedges of bone graft prop the osteotomy open.
A prerequisite for Dega osteotomy is an open triradiate cartilage. Dega osteotomy allowed excellent correction of the superior and lateral deficiency seen preoperatively. A similar osteotomy is used combined with a shortening femoral VDRO and aggressive muscle release.
Hip pain Inability to sit in a modified wheel chair. Difficulty with perineal hygiene duo to contractures
There are 4 surgical options: Proximal femoral resection. Valgus osteotomy of proximal femur. Hip arthrodesis. Total hip arthroplasty.
The femoral head is repositioned farther lateral from the acetabulum, so that the rubbing between the pelvis and the head is lessened.
Valgus osteotomy
Hip arthrodesis
Surgical fusion of the hip is been performed in patients with painful dislocations of the hip due to cerebral palsy. The desired position of fusion in non ambulatory patients is 50 degs of flexion and 10 degs of abduction.
Root used a combined intra and extra articular technique, with subtrochanteric osteotomy of the femur o allow positioning of the leg.
Patients who have excessive adductors and hip flexors release, leading to extension and abduction contracture. Children with severe neurological involvement with extension posturing.
Symptoms: inability to sit in wheelchair due to extension contracture. and pain in half of those patients. On Physical examination.. the femoral head is palpable in the anterior groin. and the hip flexion is limited. In the radiograph, the hip may appear reduced as it lies anterior to the acetabulum. Treatment: Aggressive muscle release of proximal hamstrings, and If the patient is abducted, the abductors and short external rotators must be included. Varus shortening osteotomy of the femur is recommended, in conjunction with acetabular osteotomy to gain anterior coverage. The Pemberton osteotomy is useful here.
Cerebral palsy
Spinal deformity
Scoliosis.. Spondylosis..
scoliosis
Affecting between 25-68% of patients with cerebral palsy. Incidence is highest in non ambulatory patients, and who have total body involvement. Patients with spasticity are at high risk, compared to other movement disorders.
The usual curve pattern is a long sweeping curve that extends to the pelvis. with the apex of the curve at the thoracolumbar junction. Rotation accompanies the coronal plane curvature. Scoliosis leads to difficulty in sitting, and in using wheel chair. with: pressure sores on ischium teberosities, and a hand dependent sitter.
Scoliosis..
Indications for spinal fusion are:
Curves of more than 50 degs in ambulatory patients. Progressive curves of more than 50 degs in patients who are communicative and aware of their surroundings. Curves that interfere with seating and nursing in patients whose families desire surgical correction.
Segmental fixation is preferred. The role in scoliosis due to cerebral palsy is to instrument and fuse long. The fusion extends from T2 to the pelvis in non ambulatory patients, and to the lower lumbar spine in those who walk. The Luque technique, which uses sublaminar wires at each level Distributes the corrective forces equally through the spine. Allows greater correction and better sagital contouring, And there is less loss of fixation. Fusion of the spine to the pelvis is accomplished using the Galveston technique.
Scoliosis
The Dunn McCarthy technique is suitable for many cerebral palsy patients. Two S-shaped rods are placed over the sacral ala. with upgiong laminar hooks or pedicle screws placed more proximally. then segmental fixation is completed.
The advantage is that the sacro iliac joint is not crossed, which leads to less movement and loosening over time.
The anterior release, discectomy and fusion are recommended in: Younger patients with open triradiate cartilages. Severe, stiff curves. Curves over 70 degs. In patients which a stretch spine radiograph shows lack of correction of pelvic obliquity.
Using the Luque rods and sublaminar wires with the Galveston technique.
Rhizotomy
Selective dorsal rhizotomy in which a percentage of the dorsal roots are severed at the level of cauda equina. Spasticity is improved by reducing the stimulatory inputs from the muscle spindles of the lower extremities that arrive via afferent fibers in the dorsal roots. Usually 25-50% of the posterior nerve rootlets from L2 to S2 are divided. The rate of good results following rhizotomy depends on proper patients selection Candidates are younger than 8yrs. Must have purely spastic type. Should have no fixed contracture. Must be able to ambulate without relying on spasticity for strength. There is no weakness in the antigravity muscles or trunk musculature. Must have supportive families and be able to cooperate with postoperative physical therapy.
Cerebral palsy
Fractures
Fractures
Bone mineral density is decreased in patients with cerebral palsy. Low levels of vit.D have been documented in up to 42% of children with severe cerebral palsy. Up to 74% of fractures occur in the femur ,particularly at the supracondylar level. Treatment of osteopenia:
A. Vit.D supplementation. B. Biphosphonate.
Treatment of fractures:
THE END
22nd-Aug-2006
MoKazem.com
. . , . This lecture is one of a series of lectures were prepared and presented by residents in the department of orthopedics in Damascus hospital, under the supervision of Dr. Bashar Mirali. This site is not responsible of any mistake may exist in this lecture.
Dr. Muayad Kadhim .