Lec 1 Cerebral Palsy & Spina Bifida PDF

Cerebral palsy & Myelodysplasia Dr.Sarkawt S.Kakai FKHMS (Ortho. & Trauma) Lec.1 Nonprogressive upper motor neuron disease (static encephalopathy) due to injury to immature brain Orthopedic manifestations Contractures Fractures Upper extremity deformities Hip subluxation and dislocation Spina...

Cerebral palsy & Myelodysplasia Dr.Sarkawt S.Kakai FKHMS (Ortho. & Trauma) Lec.1 Nonprogressive upper motor neuron disease (static encephalopathy) due to injury to immature brain Orthopedic manifestations Contractures Fractures Upper extremity deformities Hip subluxation and dislocation Spinal deformity Foot deformities Gait disorders Pathophysiology Patho-anatomy • leads to muscle imbalance with a mixture of weakness and spasticity • the encephalopathy is static while the affected portion of the musculoskeletal system changes with growth Risk factors • Prematurity (most common) • Anoxic injuries • Perinatal infections • toxoplasmosis • Brain malformations • Brain trauma Secondary (growth and spasticity related) • Contractures • starts as dynamic contractures, become static with time (continuous muscle contraction results in shortening) and growth (growth of bones occurs at a faster longitudinal rate than muscles in spastic cerebral palsy) • Upper extremity deformities • Hip subluxation and dislocation • Spinal deformity • Foot deformities • Gait disorders • Fractures • Primary • abnormal tone • loss of motor control • impaired balance • spasticity • (Hypotonia) • (Dyskenesias such as chorea and athetosis) Associated conditions Classification Physiological Spastic Most common Velocity-dependent increased muscle tone and hyperreflexia with slow, restricted movement due to simultaneous contraction of agonist and antagonist muscles. Most amenable to operative treatments. Athetoid Characterized by a constant succession of slow, writhing, involuntary movements Ataxic Characterized by inability to coordinate muscle movements. Results in unbalanced, wide based gait. Mixed Usually mixed spastic and athetoid features and involves the entire body Hypotonic Usually precedes spastic or ataxic for 2-3 years Anatomic Classification Quadriplegic Diplegic Hemiplegic Total body involvement and non-ambulatory Legs more than arms but usually still ambulatory. IQ may be normal (injury in brain is midline) Arms and legs on one side of the body, usually with spasticity Will eventually be able to walk, regardless of treatment Gross Motor Function Classification Scale (GMFCS) Type I Near normal gross motor function, independent ambulator Type II Walks independently, but difficulty with uneven surfaces, minimal ability to jump Type III Walks with assistive devices Type IV Severely limited walking ability, primary mobility is wheelchair Type V Non-ambulator with global involvement, dependent in all aspects of care Clinical history Evaluation 1.History • perinatal history • growth & development • prior medical treatments Functional status • • • • sitting/standing posture upper and lower extremities function communication skills acuity of hearing and vision 2.Physical Exam General musculoskeletal exam Gait Spine exam • motion, tone, and strength • Rotational limb profiles for torsional deformities • gait lab analysis • plantigrade feet • crouch • stiff knee gait • presence and flexibility of scoliosis • spinal balance and shoulder height • pelvic obliquity • resting head posture Hips • hip contractures • flexion contracture (lead to excessive lumbar lordosis) • adduction contracture • hip instability and dislocations are common, may be looked over as a contracture alone Foot and ankle • equinovarus and planovalgus deformities common • toe walking or absent heel strike during gait secondary to gastrosoleus spasticity and contracture • may hyperextend knee to obtain heel contact • Provocative Silverskiold test to differentiate gastrocnemius contracture vs achilles contracture Imaging • Radiographs • standard radiographs should include • AP and lateral of hips • standing spine radiographs as baseline • MRI Treatment spasticity & deformity Non-operative Physical therapy, bracing/orthotics, medications for spasticity Operative Spasticity control Botox (botulinum - A toxin) Baclofen Soft tissue procedures/releases Selective dorsal rhizotomy Bony procedures/deformity correction is a common group of congenital disorders caused by various chromosomal abnormalities that lead to the failure of closure of the fetal spinal cord Myelodysplasia Present with anatomic anomalies and neurological impairment of varying degree. Risk factors folate deficiency •supplementation can decrease risk by 70% maternal hyperthermia maternal diabetes Associated Conditions Orthopaedic manifestations • pathologic fractures • spine deformities • scoliosis • kyphosis • hip dysplasia • hip dislocations • contractures • knee deformities • tibial torsion • contractures • foot deformities Neurosurgical manifestations • hydrocephalus • 70% incidence • tethered cord Urological manifestations • neurologic bladder Classification Forms of Myelodysplasia Spinal bifida oculta • defect in vertebral arch with confined cord and meninges Meningocele • protruding sac without neural elements Myelomeningocele • protruding sac with neural elements Rachischisis • neural elements exposed with no covering Function level (described by lowest functioning level) Level Function L2 Nonambulatory L3 Marginal Household ambulator High risk of hip dislocation L4 Household ambulator plus Key level because quadriceps can function Primary Motion Primary Muscles Hip flexion Hip adduction Iliopsoas (lumbar plexus, femoral n.) Hip adductors (obturator n.) Knee extension Ankle dorsiflexion & inversion Quadriceps (femoral n.) Tibialis anterior (deep peroneal n.) EHL (deep peroneal n.) EDL (deep peroneal n.) Gluteus med.& min. (superior gluteal n.) L5 Community ambulator Toe dorsiflexion Hip extension Hip abduction S1 Normal ambulator Foot plantar flexion Gastroc-soleus (tibial n.) S2 Normal ambulator Toe plantar flexion FHL (tibial n.) S3,4 Normal ambulator Bowel & bladder function Imaging • Radiographs • useful for monitoring • scoliosis/kyphosis • hip dysplasia • pathologic fractures • MRI • change in neurologic exam prompts urgent MRI to rule out cord tethering Prognosis Survival and neurologic impairment depend on level of spinal segment involved Untreated infants have a mortality rate of 90-100% Ability to ambulate • L3 or above are mostly confined to a wheelchair • L5 level patients have a good prognosis for independent ambulation Parent/carer education Physiotherapy management will eventually be handed over to the parents or carers of the infant. Initially, they will be encouraged to observe the physiotherapist carrying out range of motion exercises and handling and positioning strategies before being asked to duplicate these treatments independently. Following these teaching sessions, certain roles will then be handed over to the parents and carers. • https://youtu.be/i0YpaYitEF4