1.3 Cerebral Palsy (S).pdf

OT1026 MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION SHIFT 1 Cerebral Palsy LESSON...

OT1026 MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION SHIFT 1 Cerebral Palsy LESSON Richelle Ann S. Santiano, MD, FPNA | August 30, 2024 03 Dr. Sigmund Freud: First to state that CP might be TABLE OF CONTENTS CEREBRAL PALSY caused by abnormal development before birth and is 1. Definition associated with other disorders 2. Epidemiology 3. Etiology/Risk Factors EPIDEMIOLOGY 4. Prenatal Hypoxic-Ischemic Cerebral Injury Leading cause of childhood disability 4.1. Volpe’s Subtypes of Hypoxic-Ischemic Overall prevalence rate: 2.11 per 1000 live births Neuropathology 4.1.1. Parasagittal Cerebral Injury Dramatic increases in prevalence are seen in children 4.1.2. Periventricular leukomalacia with very low birth weight (59.6 per 1000 live births) and 4.1.3. Focal and multifocal ischemic brain very early gestation (111.8 per 1000 live births) necrosis 4.1.4. Status marmoratus ETIOLOGY/RISK FACTORS 4.1.5. Selective neuronal necrosis 5. Classifications 5.1. F PRECONCEPTION (MOTHER) 6. Clinical Patterns Mother alone 6.1. Neonatal Pattern Maternal seizures, intellectual disability, thyroid disease 6.2. Diplegic Pattern (hyper and hypo), history of stillbirth or neonatal death, 6.3. Hemiplegic Pattern maternal age older than 40 years, and low socioeconomic 6.4. Quadriplegic Pattern 7. History status 8. Clinical Examination 9. Diagnosis and Workup ANTENATAL (DURING PREGNANCY) 10. Management Birth defects, small for gestational age, low birth weight, placental abnormalities, maternal disease during LEGEND pregnancy (respiratory, heart, seizures, and incompetent ★ Important / Take Note ✤ Textbook Information cervix), abnormalities in fluid volume, maternal bleeding ➤ Lecturer’s Verbatim ❐ Other Transes/Resources in the second and third trimesters, hypertension, preeclampsia, and chorioamnionitis CASE B.C is a 10-year old right-handed child born at 36 weeks INTRAPARTUM (DURING LABOR) of gestation whose brain imaging shows a prenatal stroke Birth hypoxia (lack of oxygen to the brain), meconium on the left. He has right spastic hemiplegia, treated with staining, meconium aspiration, abnormal duration of botulinum injections every 4 months. labor, and fetal presentation He is on ankle-foot orthosis to help in walking, running and playing soccer with his friends. NEONATAL (BIRTH TO 28 DAYS OF AGE) He is in regular classes at school and has no problem Prematurity, low birth weight, growth retardation, seeing, hearing, eating, talking, or socializing. seizures, respiratory distress, hypoglycemia, infections, and jaundice CEREBRAL PALSY A group of permanent disorders of the development of POSTNATAL (PAST NEONATAL STAGE) movement and posture, causing activity limitation, that Stroke, abusive head trauma, bacterial meningitis, and are attributed to nonprogressive disturbances that motor vehicle crashes occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, 70-80% are prenatal in origin. Prematurity remains the most perception, cognition, communication and behavior, common antecedent of cerebral palsy by epilepsy, and by secondary musculoskeletal problems PATHOPHYSIOLOGY A higher proportion of children had growth retardation, Immaturity, fragile brain vasculature and physical dental enamel abnormalities, and abnormal stresses of prematurity → compromise of cerebral blood dermatoglyphic patterns and other congenital anomalies flow → Intraventricular hemorrhage (IVH) or dysmorphic features Outcome of infant with IVH depends on the degree of Dr. William John Little: First person to study CP (1853) associated parenchymal injury Sir William Osler: Coined the term “cerebral palsy” UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 1 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy PRENATAL HYPOXIC-ISCHEMIC CEREBRAL INJURY SELECTIVE NEURONAL NECROSIS Associated with multiple pregnancy, maternal bleeding, Most Common variety of injury maternal drug use, or most frequently idiopathic Coexists with one or more of other lesions Lack of blood supply (ischemic) and oxygen (hypoxic) on Specific Neurons Vulnerable: hippocampus, lateral the cerebrum geniculate body, thalamus, caudate, putamen and globus pallidus, CN 5 and 7 motor nuclei VOLPE’S SUBTYPES OF HYPOXIC-ISCHEMIC NEUROPATHOLOGY Pathogenesis related to oxygen deprivation and excitotoxic amino acids PARASAGITTAL CEREBRAL INJURY Long term sequelae: mental retardation and seizures Involves bilateral cortical and adjacent subcortical white matter necrosis of the superior medial and posterior GENESIS OF HYPOXIC-ISCHEMIC EVENT aspects of the cerebral convexities Usually prenatal Most Frequent Long Term Consequence: spastic Cerebral ischemia before the 20th week of gestation → quadriplegia (because it is bilateral) neuronal migration deficit ○ In a developing brain, the nerve cells fail to migrate to where they should be located 28-34 weeks → periventricular leukomalacia 34-40 weeks → focal or multifactorial cerebral injury CLASSIFICATION Topography or distribution of affected extremities Hemiplegic – unilateral upper and lower limb distribution ○ Focal damage Quadriplegic or tetraplegia – bilateral upper and lower limb distribution PERIVENTRICULAR LEUKOMALACIA ○ Diffused damage Occurs in the preterm infant involving bilateral white Diplegic – bilateral lower limb distribution matter necrosis adjacent to the external angles of the lateral ventricles affecting the centrum semiovale and Motor sign optic and acoustic radiations Positive – involuntary increases in frequency or Long term Manifestations: spastic diplegia and spastic magnitude of muscle activity quadriplegia, with visual and cognitive deficits in more ○ Hypertonia severe injury Spasticity ○ Effects in optic and acoustic gradations Dystonia Rigidity ○ Hyperkinesia Dystonia Chorea — dance-like and uncontrollable movement Athetosis — slow writhing movement in distal extremities Myoclonus — sudden jerk or involuntary movement of muscles Tremor (rhythmic shaking), tics, and stereotypes Negative – lack of muscle activity or control FOCAL AND MULTIFOCAL ISCHEMIC BRAIN NECROSIS ○ Weakness Injury to all cellular elements caused by an infarction ○ Reduced selective motor control within a vascular distribution ○ Ataxia ○ MCA most commonly affected ○ Apraxia or Incoordination Long term neurologic manifestations: spastic hemiplegia, spastic quadriplegia, and seizures Movement patterns typically increase with stress or purposeful activity STATUS MARMORATUS Deep tendon reflexes are usually normal to slightly Rarest lesion increased and some spasticity can be present Neuronal injury within the basal ganglia Pseudobulbar symptoms such as dysarthria, swallowing Associated with one of the neuropathologic subtypes difficulty, drooling, and oromotor dyskinesias might be Long Term Neurologic Manifestation: choreoathetosis present ○ Dance-like with slow writhing movement Involuntary movements cease during sleep UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 2 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy CLINICAL PATTERNS ○ Infection (TORCH), medications, bilirubin encephalopathy Cortical Sensory Deficit NEONATAL PATTERN ○ Hemiplegia After a severe brain injury, neonates tend to be hypotonic Pulmonary and develop spasticity over time ○ Deficit ventilation It can be difficult to elicit muscle stretch reflexes, and ○ Bronchopulmonary dysplasia in premature there are minimal milestones to evaluate infants Milestones may be delayed in premature children until 2 ○ Micro-aspirations with oromotor dysfunction years FUNCTIONAL DIPLEGIC PATTERN Gross Motor Function Classification System (GMFCS) Hypertonic deficits are noted mainly in bilateral lower ○ Sitting, standing, using stairs limbs ○ 5 levels in 5 age groups Scissoring of bilateral lower limbs and talipes Manual Ability Classification System (MACS) equinovarus deformities predominate ○ UE use ○ Equinovarus — inverted/papasok foot Communication Function Classification System (CFCS) If ambulatory, toe walking with crouching tends to be Eating & Drinking Ability Classification System (EDACS) present Moderate: Definitive difficulties in ADLs; need AD and Loss of range of motion and hyperreflexia is present in bracing the lower limbs A total of 38% of children with CP will have this pattern CLASSIFICATION SYSTEM HEMIPLEGIC PATTERN Level GMFCS MACS CFCS Hypertonic focal deficits, early hand lateralization, loss of ROM in affected limbs, and asymmetric limb use are I Walks without Hands objects Sends and evident limitations easily and receives Persistent primitive reflexes may be present successfully information with familiar and A total of 39% of children with CP will have this pattern unfamiliar partners effectively and QUADRIPLEGIC PATTERN efficiently Diffuse spastic hypertonicity with truncal dystonia, II Walks with Handles most Sends and persistent primitive reflexes, intellectual disability, limitations objects but with receives hyperreflexia, and loss of ROM in all affected limbs somewhat information with A total of 23% of children with CP will have this pattern reduced quality familiar and and/or speed of unfamiliar partners achievement but may need ASSOCIATED PROBLEMS IN CEREBRAL PALSY extra time Mental retardation ○ 50% incidence, most common in rigid, atonic, III Walks using a Handles objects Sends and and severely spastic quadriplegia hand-held with difficulty receives Seizures mobility device needs help to information with prepare and/or familiar partners ○ 50% incidence; most frequent in hemiplegia and modify activities effectively, but not spastic quadriplegia with unfamiliar Oromotor partners ○ Difficulty sucking, swallowing, and chewing ○ Poor lip closure, tongue thrust, drooling, IV Self-mobility Handles a Inconsistently with limited selection sends and/or dysarthria limitations; of easily receives ○ Most common in spastic quadriplegia and may use managed information even dyskinetic powered objects in with familiar Gastrointestinal mobility adapted partners ○ Reflux, constipation situations Dental V Transported in Does not handle Seldon effectively ○ Enamel dysgenesis, malocclusion, caries, a manual objects with sends and gingival hyperplasia wheelchair severely limited receives Visual ability to perform information even ○ Strabismus, refractive errors even simple with familiar ○ Hemianopsia in hemiplegia actions partners Hearing impairment UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 3 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy CLASSIFICATION GAIT ASSESSMENT Multifaceted classification system proposed in 2006: Hip — observe for excessive hip flexion, hip adduction motor abnormalities, accompanying impairments, and femoral anteversion anatomic and neuroimaging findings, and causation and Knee — note flexion and extension along with varus and timing valgus stress World Health Organization (WHO), in collaboration with Foot — look for equinus or toe walking along with many organizations (2007) : International Classification of dynamic varus or valgus of hindfoot Functioning, Disability and Health for Children and Youth (ICF-CY) HIP ASSESSMENT ○ Internationally accepted terminology and Tests for hip contracture classifications that focus on the relationship Thomas test between structure and function and moves from ○ Bringing both legs up to the chest to stabilize the medical model of disability to a biopsychosocial lumbar spine model of care ○ One leg at a time is extended until there is resistance in hip extension or movement in the INT’L CLASSIFICATION OF FUNCTIONING, DISABILITY, & HEALTH pelvis Broken down into 5 categories: body function (b), body ○ Measurement taken by angle of the femur and structure (s), activity and participation (d), environmental the table factors (e), and personal factors Ely test Each domain has multiple levels associated with it: ○ Done in a prone position with examiner’s hand chapter number (first digit), second-level categories (two on the buttock digits), third- level categories (one digit), fourth-level ○ Lower leg is quickly flexed, and if buttock rises categories (one digit), and qualifiers (one digit) off the table à spastic or tight quadriceps Staheli test ○ Done with the patient in a prone position with the Level Example Coding legs dangling over the edge of the table Category Body function b ○ Pelvis is stabilized, then one leg at a time is extended until there is anterior movement of the Chapter Chapter 2: Sensory b2 pelvis functions and pain ○ Angle is measured by the femur and a horizontal line Second level Seeing functions b 2 10 Test for Adductor Contracture Third level Quality of vision b 2 10 2 Test for Internal and External Rotation Fourth level Color vision b 2 10 2 1 HISTORY Medical history, birth history, family history of blood clots or stroke, and any possible risk factors for CP Developmental history should include when and how a milestone wa achieved as well as if there is regression of milestones after attainment Functional history ○ Discussing the individual’s mobility in the home and community setting, education, participation in ADLs and participation in community activities ○ Equipment CLINICAL EXAMINATION MUSCULOSKELETAL EXAMINATION Static ○ Isolating joint movements and passive ROM Dynamic ○ Movement and function UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 4 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy EVALUATION OF THE POSTERIOR TIBIALIS AND PERONEAL MM Spastic posterior tibialis can cause varus of the heel along the plantar flexion LEG LENGTH EVALUATION Spastic peroneus can cause valgus deformity. Should be taken from anterior-superior iliac spine to the medial malleolus TEST FOR TIBIAL TORSION Knee contractures: 2 segments – medial joint space in the knee as an additional anatomic point Prone: Thigh-foot angle Pelvifemoral angles are asymmetric – measurements Sitting: Femoral condyles lined up, line is drawn through are from the greater trochanter to the medial malleolus the malleoli KNEE ASSESSMENT BACK ASSESSMENT Test for Hamstring contracture ○ Popliteal angle is measured by stabilizing the UPPER EXTREMITY ASSESSMENT contralateral leg on the table, then flexing the ipsilateral hip to 90 degrees NEUROLOGIC EXAMINATION ○ Lower leg is extended until resistance is felt Tone ○ Angle is measured from either the tibia and the Postural and reflex assessment line of full extension or the 90 degree position to full extension DIAGNOSIS Evaluation of Position of Patella The term CP does not reflect a specific etiology but is a Test for Posterior Capsular Tightness description of clinical findings and a diagnosis of ○ Done with the legs extended on the examination exclusion table The initial evaluation of a child with suspected CP should ○ Knees extended until there is resistance determine the diagnosis, decide which tests are needed to make the diagnosis; determine the etiology of the symptoms; determine comorbidities; and develop treatment options LABORATORY TESTS AND DIAGNOSTIC IMAGING Metabolic and genetic diseases: Thyroid functioning, lactate and pyruvate, organic and amino acids, and chromosomes Perinatal asphyxia ○ Blood pH ○ CSF protein ○ Lactate-to-pyruvate ratio Cranial ultrasound Popliteal Angle Test ○ Premature infant ○ To check for intraventricular hemorrhage FOOT AND ANKLE ASSESSMENT Cranial CT Test for Gastrocnemius/Soleus Contracture Cranial MRI ○ Silfverskiold test of ankle dorsiflexion ○ Most beneficial after the child is 2-3 weeks PET ○ Define blood flow and glucose metabolism SPECT ○ Document cerebral perfusion UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 5 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy MR spectroscopy ○ Series of set patterns repeated many times ○ Compares MR signal ratios of phosphocreatine during the day, attempting to train cerebral to inorganic phosphorus to find indications of dominance and normalization of function cerebral asphyxia Rood Evoked potentials ○ Activating muscles through sensory receptors ○ Useful in assessing anatomic pathways of Bobath and Bobath auditory and visual pathways ○ Normalize tone, inhibit abnormal primitive reflex EEG patterns, and facilitate automatic reactions and ○ For comorbid seizures subsequent normal movement ○ Providing key points of control throughout the body Vojta ○ Activation of postural development & equilibrium reactions to guide normal development Conductive education ○ To function independently in the world w/o aids FUNCTIONAL TRAINING Easier to prevent tightness by repeated activities than to correct it after it has developed MANAGEMENT Stretching to increase motion should be slow and gentle with child relaxed THERAPEUTIC MANAGEMENT Increase strength à progressive resistive exercise and Early intervention isometric and isokinetic training can be used Therapeutic exercise Younger child –age-appropriate play, adaptive toys and Phelps games ○ Extensive bracing, withdrawing support as Training of postural and motor control should follow motion is performed with a minimum of tension, developmental sequence overflow, and substitution Training of balance should progress from symmetric to ○ Emphasis on self-help skills and development of asymmetric patterns balance and position sense To develop optimal motor performance, individual Deaver voluntary motor control must be established ○ Extensive bracing, limiting all but two motions of Once basic control has been established, therapy can be an extremity directed toward coordination ○ Voluntary motion was emphasized for ADL Gross motor abilities and hand dexterity are physical performance determinants for planning a program in ADLs Temple, Fay, Doman and Delacato UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 6 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy THERAPY INTERVENTIONS TRANSCRANIAL MAGNETIC AND DIRECT CURRENT Main goal of all therapies is to promote and facilitate STIMULATION development in all domains of function Transcranial magnetic stimulation (TMS) and transcranial Therapies are commonly provided at school, home, and direct current stimulation (tDCS) are both experimental in out-patient clinics with a frequency of one to two times interventions that are showing potential benefit in both a week per discipline children with CP Regardless of the setting, it is imperative that the family Small studies have reported improved “proprioception, and child are included in the therapy plan to promote mobility, body sway and balance, gait distance and carryover and reinforcement in the home setting velocity, and spasticity” over weeks to months particularly with tDCS alone or in combination with standard STRETCHING therapies Goal is to reduce the risk of contracture development as a result of muscular imbalance and hypertonicity ROBOTIC AND PARTIAL BODY WEIGHT-SUPPORTED TREADMILL Sustained stretching can be achieved through the use of TRAINING positioning devices, orthoses, and serial casting These forms of therapy involve a repetitive and task A 2017 Cochrane systematic review found high-quality specific approach to facilitate attainment of stepping and evidence that “stretch did not have clinically important locomotion and to achieve a more normalized gait pattern effects on joint mobility” in individuals with neurologic based on current theories of motor learning conditions. However, no study followed patients longer The setting includes an overhead harness system used to than 7 months, and there was no conclusive evidence if support the child’s body weight on a treadmill, while the stretching affects quality of life or pain therapist or a robot facilitates the kinematic, kinetic, and temporal features of walking STRENGTHENING AND AEROBIC EXERCISE Several systematic reviews showed limited evidence to A systematic review of the effectiveness of strength support the use of PBWSTT in children with CP training programs in CP reported increased strength without any negative effects of increased spasticity or ORTHOSES AND DURABLE MEDICAL EQUIPMENT reduced ROM Goals: prevent contracture, provide optimal joint Indirectly some studies have reported increased alignment, provide selective motion, protect weak participation and improved self-esteem muscles, control abnormal tone and related deviations, A systematic review of the evidence for aerobic exercise enhance function and protect tissues postoperatively suggests improved physiologic measures of aerobic Ankle-foot orthosis – most frequently prescribed braces fitness without adverse effects such as increased ○ Control spastic equinus spasticity, fatigue, or musculoskeletal trauma ○ Promote alignment of hindfoot Improvements in aerobic fitness were noted with 45 ○ Control midfoot and excessive knee extension in minutes four times a week of “high intensity” activities stance such as wheelchair sports, swimming, mat exercises, or Wheelchairs and mobility aids cycling Supportive or adaptive seating systems and standing frames CONSTRAINT-INDUCED MOVEMENT THERAPY Computers, switch devices, sign boards Treatment for hemiparesis to improve motor function in the affected upper limb In children with hemiplegic CP, the unaffected limb is MANAGEMENT OF SPASTICITY restrained with a plaster or removable cast, typically for 3 Therapeutic exercise – ROMs exercises, casting and weeks, and the child undergoes intensive, structured splinting, functional electrical stimulation therapy in addition to daily activities and play ○ Casting for 2-3 weeks can improve tone FUNCTIONAL ELECTRICAL STIMULATION ORAL MEDICATION FOR HYPERTONIA Neuromuscular electrical stimulation (NMES) is the Baclofen, diazepam, dantrolene sodium, tizanidine, application of an electrical current of sufficient intensity to clonazepam, and clonidine elicit muscle contraction ○ Be careful in giving these medications as you When applied during functional activity, it is referred to as are giving meds to pediatric clients → be aware functional electrical stimulation (FES) of the side effects All can cause sedation, except dantrolene, as they exert their effects through the CNS Pediatric dosing is quite variable, and the side effects and risks of these medications limit their use UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 7 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy The treatment of generalized secondary dystonia in NEUROLYSIS WITH ALCOHOLS children with CP is more challenging because it responds Alcohol works by proteolytically causing neurolysis poorly to oral medications The use of alcohol requires isolation of the desired nerve Commonly used drugs include oral baclofen, and with electrical stimulation with or without ultrasound levodopa-carbidopa guidance, which can be technically challenging Adverse events such as sedation, dizziness, and dry The need for electrical stimulation also adds an additional mouth are common and can limit its tolerance element of discomfort to the patient. For this reason, However, intrathecal baclofen has been found to be more sedation is highly recommended effective at higher doses than what is required for Commonly injected nerves are the musculocutaneous, treatment of spasticity obturator, and sciatic nerve to the medial hamstring In contrast to BoNT-A, alcohols are inexpensive and the results of neurolysis can last between 6 and 18 months BoNT-A and BoNT-B have been combined safely with alcohol injections, thus allowing one to maximize the dose and number of muscles treated by the finite amount of BoNT INTRATHECAL BACLOFEN THERAPY ITB is most often used to treat children with generalized spasticity or generalized moderate to severe dystonia Currently, indications for the use of ITB therapy include tone that is thought to interfere with function or the ability to provide care; modified Ashworth scores of greater than 3; and definable goals for spasticity reduction ITB is delivered through a programmable pump placed subfascially in the abdominal wall and connected to a catheter that is tunneled from the side of the pump and inserted in the intrathecal space at a desired spinal level This method allows the delivery of smaller doses of baclofen (micrograms) intrathecally, thus reducing the side effect profile seen with the oral form of baclofen FOCAL INTERVENTIONS (milligrams) Botulinum toxin (BoNT) intramuscular injections and alcohol (ethanol or phenol) neurolysis are used in the DEEP BRAIN STIMULATION treatment of focal hypertonicity (spasticity and dystonia) Involves as stereotactic implantation of electrodes into Injections are most effective as a localized treatment for the basal ganglia and a programmable pulse generator dynamic contractures or shortened muscles implanted subcutaneously in the infraclavicular region Chemodenervation and neurolysis can be used Treatment of choice in primary dystonias and is often simultaneously to maximize the benefits by allowing for used in people with CP with generalized secondary higher concentration of BoNT in target muscles and dystonia (typically quadriplegics with GMFCS levels IV-V) weakening of a larger number of target muscles In those who do not respond adequately to ITB, DBS ○ Every 3-4 months ultrasound-guided injection should be considered CHEMODENERVATION WITH BOTULINUM TOXIN (BONT) SELECTIVE DORSAL RHIZOTOMY BoNT works by presynaptically blocking release of Surgical procedure used as a treatment for spasticity acetylcholine into the neuromuscular junction Single or multilevel laminectomies exposing the L2–S2 Botox and Dysport (commercial preparations of BoNT-A) nerve roots and selectively cutting a percentage of the are US FDA-approved for use in children and adults with dorsal rootlets with abnormal response with the aid of CP electrophysiologic monitoring The period of clinically useful relaxation is usually 3 to 4 The ideal candidate for an SDR is a child months, and it is recommended that injections be spaced betweentheagesof3and8yearsof age with spastic diplegic a minimum of 3 months apart due to concern for CP, typically GMFCS levels I-III, little upper limb neutralizing antibody formation involvement, sufficient underlying strength, good selective Adverse events related to BoNT-A are rare and include motor control, and minimal contractures injection site pain, fatigue, and excessive weakness of Positive preoperative functional predictors foragood SDR the injected muscle outcome include the ability to rise from a squatted Some cases of dysphagia and urinary incontinence have position with minimal support and a younger child’s ability been reported to crawl on hands and knees or tall kneel UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 8 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy ORTHOPEDIC MANAGEMENT Scoliosis surgery may improve restrictive lung disease Surgeries designed to improve ambulation are preferred but must be considered carefully in the context of whether in patients at GMFCS levels I-III, whereas surgeries surgery will improve functioning and quality of life aimed toward improving care and comfort are performed in those with GMFCS levels IV-V NEUROLOGIC ISSUES Orthopedic surgical procedures should be considered in The CP population is at an increased risk for seizures the presence of contractures that hinder function or (∼30%), most frequently in children with quadriparetic or interfere with hygiene, subluxation/dislocation of joints hemiparetic clinical presentation (most commonly the hips), and rotational problems or Children with CP also have a higher incidence of lever arm dysfunction (LAD), causing gait problems intellectual disability, with cognitive impairment estimated Four major types of orthopedic surgeries in CP: muscle to be 50% release and lengthening, tendon transfer, osteotomy, and Nonverbal status and severe intellectual disability are arthrodesis seen more commonly in children with a quadriparetic Any orthopedic surgical intervention should be delayed clinical presentation until ages 7 to 9 years due to a high risk of recurrence, Screening for impaired attention and Executive unless there is evidence of hip subluxation functioning deficits is recommended Some children will be at higher risk for vision and hearing FEEDING, GROWTH, AND NUTRITION deficits Dysphagia is commonly observed in the CP population Prematurity is associated with retinal damage and myopia ○ At risk for not meeting caloric and fluid Children with dyskinetic CP due to kernicterus should be requirements due to feeding inefficiency screened specifically for sensorineural hearing loss ○ Malnutrition And Increased Risk For Aspiration ○ Oral Hygiene may be compromised, and GENITOURINARY children may have excessive sialorrhea Voiding dysfunction may result from impaired processing ○ Growth and nutrition may be compromised due of sensory feedback and also incoordination of muscular to limited intake of key nutrients, impaired functions responsible for bladder wall contractions and absorption, and endocrine abnormalities sphincter relaxation, detrusor sphincter dyssynergia Children when malnourished are at risk for growth Urinary retention can increase chances of urinary tract disturbances, increased infection rates, skin breakdown, infections, and if a high intravesical pressure is and osteopenia and ultimately may have a decreased life generated, vesicoureteral reflux with resultant expectancy hydronephrosis and pelviectasis may occur Anticholinergic treatments for dystonia or sialorrhea may PULMONARY trigger urinary retention Lung disease is an important cause of morbidity and Children with levetiracetam use for epilepsy have an mortality in CP increased risk of renal calculi formation ○ Assess for high-risk of aspiration if difficulty with swallowing GASTROINTESTINAL Prevention of pulmonary aspiration may require modified Gastroesophageal reflux disease (GERD) and feeding consistencies and treatment of reflux, alternative constipation, which are commonly observed in CP, may means of feeding such as gastrostomy and control of produce discomfort and exacerbate hypertonicity sialorrhea with anticholinergic medications, botulinum In more severe cases, children may have dysmotility with injections, and sometimes surgery delayed gastric emptying and esophagitis from medically Chest physiotherapy and bronchodilators can assist with refractory GERD decreased mucociliary clearance Infections should receive tailored antibiotics based on MUSCULOSKELETAL PAIN AND OSTEOPENIA sputum cultures Children who are less mobile are at increased risk for Immunizations need to be given to the CP population contractures, bony deformities, and associated because they are at a higher risk for infections and musculoskeletal pain complications Children with CP are at risk for osteopenia and related Upper airway obstruction may require continuous positive fragility fractures airway pressure (CPAP) and/or surgery Risk factors for osteoporosis include decreased weight Lower airway obstruction may respond to a trial of bearing, use of anticonvulsants, malnutrition, and bronchodilators and pressure decreased sunlight exposure Hypoventilation is seen frequently in children who have Supplementation with calcium and vitamin D is routinely neuromuscular weakness and children may ultimately used to improve bone density, but the effects on require noninvasive ventilator support and external aids prevention of fragility fractures is unclear for mobilization of secretions and to assist with cough Bisphosphonates is usually reserved for children who generation have experienced a fragility fracture UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 9 OT1026 SHIFT #1 | LESSON #2 | Cerebral Palsy COMPLEMENTARY AND ALTERNATIVE MEDICINE More evidence is required before recommendations can be made CHILDHOOD DISABILITIES AND EDUCATION Early intervention programs are government-sponsored programs that are tasked to evaluate children at risk or with delays in one or more areas of function between the ages of 0-36 months Services provided include therapies, nutrition monitoring, care coordination, audiovisual, and social work services, among others After 36 months, children with disabilities may qualify to receive ongoing services through their local public school system PSYCHOSOCIAL ISSUES To parents: ○ Do not over-protect a child, let the child learn his or her limitations ○ Be honest with the child ○ Set realistic goals for the child ○ Let the child make choices ○ Use discipline and encourage self-esteem ○ Cheer the child on TRANSITION TO ADULTHOOD AND AGING WITH CEREBRAL PALSY A child must be encouraged to become as independent as possible and to separate from caregivers in a developmentally appropriate fashion during the teenage years Children with significant cognitive and physical delays may require long-term support but may enjoy opportunities for socialization with peers other than their primary caregivers Children with disabilities may need encouragement and training on self-advocacy Vocational rehabilitation to educate and train disabled individuals to compete in the work environment FUNCTIONAL PROGNOSIS It is intuitive that the more severe the motor disorder or higher GMFCS level, the greater likelihood that the child will have more numerous or more severe medical comorbidities. Two exceptions to this correlate include pain (occurring in all levels of disability) and behavioral disorders (seen more commonly in cases of milder motor disability) About 90% of children with CP survive to adulthood Positive prognostic factors: ○ regular schooling, ○ completion of secondary school, ○ independence in mobility and ability to travel beyond home, ○ good hand skills, ○ living in a small community and ○ having spasticity as the motor dysfunction UST OT 2026 | MEDICAL-SURGICAL FOUNDATIONS FOR ADULT PHYSICAL DYSFUNCTION 10