Cerebral palsy diagnosis and management: the state of the art

Cerebral Palsy Diagnosis and Management: The State of the Art Nancy Murphy, MDa and Teresa Such-Neibar, DOb

Definition of Cerebral Palsy erebral palsy, or static encephalopathy, is defined as a primary abnormality of movement and posture secondary to a nonprogressive lesion of a developing brain. It actually represents a group of disorders rather than a single disease entity.1 Abnormal motor control and tone in the absence of an underlying progressive disease is the clinical hallmark of cerebral palsy. By definition, all neurodegenerative and metabolic conditions, which may initially mimic the disorder, are excluded from this diagnostic category. In 1862, William James Little, an orthopedic surgeon, provided the first description of “spastic rigidity” related to prematurity and birth complications, referring to the condition as Little’s disease.2 William Osler later introduced the term “cerebral palsy” in 1888. Subsequently, Sigmund Freud observed that antepartum and postpartum factors might be causally related to cerebral palsy. Since the earliest definition of cerebral palsy, many others have attempted to establish a unified description of the disorder.3 Controversy and confusion result when we attempt to relate causal factors to the physical and functional characteristics of cerebral palsy. Currently, cerebral palsy has been described as an “umbrella term” to refer to children with a wide range of static cerebral disorders with associated motor impairment.4 Inclusion and exclusion criteria useful for making the diagnosis of cerebral palsy have been written for the purpose of maintaining disease registries, such as the Western Australian Cerebral Palsy

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From the Departments of aPediatrics and bPhysical Medicine and Rehabilitation, University of Utah, Salt Lake City, UT. Curr Probl Pediatr Adolesc Health Care 2003;33:146-169. © 2003 Mosby, Inc. All rights reserved. 1538-5442/2003 $30.00 ⫹ 0 doi:10.1016/S1538-5442(03)00002-6

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Register.4 Before the availability of modern laboratory techniques, some partial trisomies, chromosomal deletions, and x-linked hydrocephalus syndrome were included in the diagnosis of cerebral palsy. Although it is recommended that these nonprogressive cerebral abnormalities associated with motor impairments continue to be included in the diagnostic grouping of cerebral palsy, consistency among practitioners is lacking. It is well recognized that very low birth weight infants are at risk for cerebral palsy. Recent attention has focused on the outcomes of very low birth weight infants who survived the neonatal period without severe intraventricular hemorrhage or periventicular leukomalacia. It is estimated that the academic achievement of 30% to 50% of these children falls in the subnormal range. Furthermore, attention-deficit hyperactivity disorder is diagnosed in 20% to 30% and psychiatric disorders in 25% to 30% by adolescence.5 Perhaps the vulnerable premature brain is harmed during a critical period of development by stressful environmental conditions, including infant-provider interactions, constant noise, and bright lights. The impact of chronic lung disease, recurrent apnea and bradycardia, hyperbilirubinemia, and other physiologic stressors on the developing infant are poorly understood. However, if these at-risk children do not have abnormalities of motor function, they are not included in the diagnostic grouping of cerebral palsy.5

Epidemiology Cerebral palsy has an estimated prevalence rate of 1.0 to 2.3 per 1000 live births. The incidence is strongly associated with gestational age. Cerebral palsy occurs in 1 of 20 surviving infants with extreme prematurity. Among infants with a birth weight of ⬎2500 g, the occurrence rate is less than 1 in 1000.6 It is important to note that although prematurity is the most common antecedent of cerebral palsy, the major-

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ity of children who develop the disorder have had full gestational term. This paradox can be explained by the fact that there are 7 to 10 times more full-term than preterm babies born.7 Despite the reduction in the rate of asphyxial births from 40 per 100,000 in 1979 to 11 per 100,000 in 1996, neither the prevalence of cerebral palsy nor the incidence of the disorder among term infants has changed during that same time period.6 Between 1960 and 1986, however, it is estimated that the childhood prevalence of cerebral palsy in the United States increased by 20%, from 1.9 to 2.3 per 1000 live births, paralleling the change in the survival of low (⬍2500 g) and very low birth weight (⬍1500 g) infants.8 In addition to the increasing prevalence of cerebral palsy among preterm infants, there is also an associated increase in the severity of disability.9 This emphasizes the importance of ongoing efforts to decrease rates of premature birth and to decrease the occurrence of neurologic injury among premature infants.8

Cerebral Palsy Subtypes Distinguishing among subtypes of cerebral palsy provides specificity to a broad diagnostic grouping. The topographic distribution of limb involvement and the quality of the movement disorder determine subtypes. It is important to note that functional impairments and degrees of disability can vary widely within subtypes. Monoplegia refers to single limb involvement. Diplegia is present when primarily the lower extremities are affected, although upper extremities are not completely spared. Hemiplegia is characterized by the involvement of one side of the body, with the arm typically more affected than the leg. When all four limbs are involved, quadriplegia is the appropriate descriptive term. Infrequently, children are diagnosed with double hemiplegic or triplegic cerebral palsy. Although each of these distributions is readily defined, it is often challenging clinically to assign some children with cerebral palsy to specific subtypes.10 Often there are inconsistencies between examiners and variations in the subtype determined by the same examiner at different times. The neurologic picture evolves as the child develops. It is not unusual for an infant with hypotonicity to become a toddler with spasticity.11 After the distribution of limb involvement is established, the quality of muscle tone and involuntary

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movement is assessed. Resting muscle tone can be described as low, normal, or high and is determined by the amount of resistance felt to slow, passive movement of the limb(s). Hyperreflexia and clonus characterize spasticity, which is defined as a velocity-dependent increase in tone. Spasticity is the most commonly occurring feature of cerebral palsy, accounting for approximately 75% of the children affected.10 A small percentage of children with cerebral palsy demonstrate extrapyramidal (dyskinetic) features, including combinations of athetosis, chorea, dystonia, and ataxia. When a child displays a combination of features, such as spasticity with choreoathetosis, mixed-type cerebral palsy is present. Hypotonic cerebral palsy does occur, but in the majority of children, there is a progression to other subtypes over time.

Spastic Quadriplegic Cerebral Palsy Spastic quadriplegic cerebral palsy is the most disabling of all types combined, with 25% of the affected children requiring total care.1 Opisthotonic posturing and the display of strong and sustained primitive reflexes during infancy are often precursors of spastic quadriplegic cerebral palsy. Affected children frequently demonstrate oral motor dysfunction and are at high risk for aspiration events. The ability to sit independently at 2 years of age is predictive of future ambulation, whereas the inability to do so by 4 years is a poor prognostic indicator for ambulation. Only one third of children with spastic quadriplegia ambulate.12 The child with persistent asymmetric tonic neck reflex, extensor thrust, and lack of parachute reflex is unlikely to walk. Overall, children with spastic quadriplegic cerebral palsy have greater functional impairments and secondary medical complications when compared with children with other types of cerebral palsy.1

Spastic Hemiplegic Cerebral Palsy Children with spastic hemiplegic cerebral palsy generally have asymmetric limb use by 6 months of age and hand preference during the first year. Their preferred means of floor mobility may involve hitching on the buttocks with assisted forward propulsion with the unaffected upper extremity.10 Over the years, growth retardation of the affected side becomes apparent. Nearly all children with hemiplegic cerebral palsy learn to walk by 2 years of age, with the gait characterized by unilateral flexion synergy of the upper extremity and extension synergy of the lower

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extremity.1 These children may also have hemianopsia, and regular ophthalmologic examinations are indicated.

Spastic Diplegic Cerebral Palsy Spastic diplegia is the type of cerebral palsy most commonly associated with preterm births. The watershed zones of the germinal matrix in the brains of preterm infants are particularly susceptible to bleeding and ischemic injury. The immediate periventricular white matter contains fibers of the pyramidal tracts of the internal capsule that descend to the spinal cord and provide neuromotor control to the lower extremities. More peripheral in the periventricular white matter are the pyramidal tracts of the upper extremities. Thus, intraventricular bleeding in preterm infants is associated with spastic diplegic cerebral palsy, affecting motor tone and control in the lower extremities more than the upper extremities. More than 50% of affected children learn to walk by 3 years of age, often with flexed and adducted hips and knees and the assistance of orthoses and ambulation aids such as walkers and crutches.1

Extrapyramidal Cerebral Palsy Children with extrapyramidal cerebral palsy begin to have distinct movement disorders between 12 and 18 months of age, characterized by athetoid and dystonic posturing. The uncontrolled movements become most apparent during volitional motor activities. Speech impairments may be severe, characterized by dysarthria, drooling, poor initiation of vocalization, and occasionally explosive utterances.10 Intelligence is normal in 78% of the children with extrapyramidal cerebral palsy, and the abilities of these children can be underestimated due to the severity of motor and communication skills. Kernicterus is a leading cause of this type cerebral palsy, as the basal ganglia and auditory nuclei are particularly susceptible to the toxic effects of bilirubin. The affected neonate appears weak, listless, and hypotonic and demonstrates poor sucking and feeding skills. Over a period of months, hypertonia, opisthotonus, chroeoathetosis, and sensorineural hearing loss develop. Infants who have the longest initial periods of hypotonicity tend to have the most significant degrees of disability.10

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Subtypes of Cerebral Palsy Subtypes of limb involvement are monoplegia, diplegia, hemiplegia, and quadriplegia; quality of movement can be spastic, extrapyramidal, or mixed.

Risk Factors Parents of a child with cerebral palsy are often eager to understand the cause of the disorder. However, the underlying cause of cerebral palsy remains unclear in more than 50% of cases.7 This strengthens the assumption that its origin probably is multifactorial. Adverse factors, such as abnormal fetal brain or environment, may make the infant more vulnerable to preterm birth, or if born at term, at greater risk for cardiopulmonary problems in the neonatal period.7 Based on findings of the National Collaborative Perinatal Project, the leading predictors of cerebral palsy include maternal mental retardation, birth weight ⬍2001 g, and fetal malformation.13 Interestingly, the study also reported that maternal age, parity, socioeconomic status, smoking history, maternal diabetes, and duration of labor were not found to be risk factors.14 The death of one twin in utero increases the risk for cerebral palsy in the surviving twin by more than 100 times.15 Cerebral palsy in term infants of normal birth weight is typically related to prenatal factors, such as cerebral malformations, prenatal strokes, and intrauterine TORCH infections. Perinatal asphyxia is believed to be the causative factor in only 8% to 10% of all cases.16 Although a normal cord pH excludes hypoxic encephalopathy, a pH of ⬍7.0 is associated with encephalopathy in only 10% to 20% of infants.2 Similarly, APGAR scores are not sensitive predictors of neurologic outcome. An APGAR score of 3 or less at 10 to 15 minutes is associated with a 10% to 15% cerebral palsy occurrence rate.17 Chorioamnionitis has been shown in a recent meta-analysis to be a risk factor for both cerebral palsy and cystic periventricular leukomalacia (PVL).18 The existence of PVL is the strongest and most independent risk factor for the subsequent development of cerebral palsy. In very low birth weight infants, ultrasonographic abnormalities of persistent ventricular enlargement or persistent parenchymal echodensities carry a 50% risk for cerebral palsy, and large bilateral cysts in the periventricular white matter carry a risk of 75% to 95%.17 Cerebral palsy occurs in 56% of infants with PVL and intraventricular hemor-

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rhage with ventricular distention, with 72% of these infants having the spastic diplegic subtype.19 Infants with cystic lesions have higher rates of quadriplegia and greater severity of disabilities.20

Making the Diagnosis Cerebral palsy is a descriptive term, based on clinical observation, rather than a diagnosis informative about etiologic factors, pathology, or prognosis.21 Among the 5.2 per 1000 children diagnosed with cerebral palsy at 12 months of age, the incidence of cerebral palsy at 7 years of age was found to be only 2 per 1000 live births.22 Thus, diagnosing cerebral palsy during the earliest years of life is often unreliable. Early warning signs include delay in meeting motor milestones, toe walking, persistent fisting, decreased rate of head circumference growth, seizures, irritability, poor suck, handedness before 2 years of age, and scissoring of the lower extremities.23 Serial neurologic, developmental, and functional assessments provide the clinician with the important diagnostic information.24 Evidence of disease progression must be continually sought, and when present, excludes the diagnosis of cerebral palsy. The persistence of primitive reflexes and delay or failure to acquire postural reactions are early indicators of central nervous system dysfunction. Primitive reflexes are mediated at a subcortical level location in the brain stem. The development of cortical connections gradually overrides these primitive responses during the first 6 to 8 months of life. While primitive reflexes are being integrated, the righting, protection, and equilibrium postural reactions are emerging. This transition may be delayed or may never occur in infants with brain abnormalities.24 Obligatory primitive reflexes at any age are abnormal. A comprehensive history for risk factors and genetic background, complete physical and neurologic examinations, and serial developmental assessments provide invaluable information when making the determination of cerebral palsy in a young child. Although ankle clonus is not uncommon during the first year, sustained clonus at any age is suspicious. An interdisciplinary approach to making the diagnosis is recommended and may necessitate input from the ophthalmologist, audiologist, radiologist, neurologist, geneticist, and child development teams. Additional evidence can be obtained from relevant metabolic, chromosomal, and neuroimaging studies.

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Parents of preterm infants are universally eager to know if their child will have cerebral palsy and often seek anticipatory guidance. Currently, the best available predictor of cerebral palsy among high-risk infants is the presence of echodensities and cysts in the periventricular white matter regions of the brain. Among infants with a birth weight ⬍1500 g, there is a 15% to 20% risk of cerebral palsy. Lorenz and colleagues found that 20% to 25% of the survivors of extreme prematurity (ⱕ26 weeks) will have at least one major disability, including mental deficiencies, cerebral palsy, blindness, or deafness.25 A metaanalysis of 111 outcome studies of VLBW (⬍1500 g) infants born since 1960 concludes, “No agreement exists about the definition of study populations, descriptive statistics, or measurements of outcome.”26 Parents therefore need to know that during the early years, we can offer only our best guess and ongoing evaluations.

Clinical Scenario: The Growing Preemie (0 to 36 Months) A 17-month old boy, born at 27 weeks’ gestation, is unable to sit independently, keeps his right hand persistently fisted, and remains exclusively dependent on bottle-feeding for nutritional support. His mother asks you if he has cerebral palsy and about the value of therapeutic interventions.

The Pediatrician’s Role The primary care pediatrician caring for the child with cerebral palsy should provide well-child care and health maintenance visits as they would for any other child, including the provision of anticipatory guidance, developmental assessments, and immunizations. Additionally, the child’s respiratory status should be carefully assessed, as bronchopulmonary dysplasia, reactive airway disease, trachomalacia, and functional upper airway obstructions are not uncommon.27 The tendency to offer only problem-focused office visits because of the many medical issues needs to be avoided. The pediatrician should discuss advanced directives throughout the lifespan with each family that includes a child with chronic illness, involving the child in the decision-making process whenever possible. All children, with or without cerebral palsy, should receive immunizations according to the recommenda-

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tions of the American Academy of Pediatrics.28 Although the MMR vaccine presents a slightly increased risk for seizures in children with known epilepsy, there is no evidence of serious acute or chronic sequelae from such seizures. Although the use of DTP has been accused of causing or exacerbating neurologic injury or altering the prognosis of children with neurologic disorders, no scientific evidence supports these claims. Furthermore, pertussis itself can lead to seizures, pneumonia, apnea, encephalopathy, and death. The AAP has recommended that DTP be withheld in children with undefined or possibly progressive neurologic disorders and that children with static neurologic conditions receive the immunizations on schedule.29 Annual influenza vaccination should be provided for all children with cerebral palsy who contend with recurrent or chronic respiratory illnesses. Pneumococcal immunization is recommended for those who have chronic or recurrent pulmonary illnesses, and particularly for those at risk for infection with antibiotic resistant organisms, such as children in long-term care facilities and residential settings.30 Sensory Impairments. Each infant born prematurely is at risk for sensory neural hearing loss caused by antibiotic treatment and neurologic dysfunction or other factors, and therefore brainstem auditory-evoked responses should be performed before or shortly after discharge from the neonatal intensive care unit. Unrecognized hearing impairments can interfere with language acquisition, thereby contributing further to developmental delays. Similarly, growing preemies are at risk for visual impairment. Retinopathy of prematurity, resulting from vascular damage to the retina, can lead to nearsightedness, strabismus, glaucoma, and blindness.31 Cortical visual impairments result from damage to the visual cortex of the occipital lobes. Visually evoked potentials assess integrity of the pathway from the optic nerve to the visual cortex but cannot assess visual acuity. Serial ophthalmologic examinations are encouraged. Seizures. The overall prevalence of epilepsy in children with cerebral palsy is 36%, with onset in the first year of life in 70%.32 Focal and generalized seizures are most common. Epilepsy can be an indicator of the severity of neurologic injury, as seizures are more common in the children with cerebral palsy associated with mental retardation and poor motor development.33 Children with congenital anomalies and term-type injuries have a higher incidence and

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earlier onset of epilepsy when compared with those with perinatal insults or prematurity.33 One study revealed that in 75% of the children with cerebral palsy, the anti-epileptic medications were discontinued after a 3-year seizure-free interval, although 13.4% of children relapsed and treatment was resumed.32

Therapeutic Interventions Neurodevelopmental Treatment. Neurodevelopmental treatment (NDT) was initially described by the Bobaths in 1964. This treatment emphasizes underlying component skills (muscle tone, reflexes, abnormal movement patterns, postural control, sensation, perception, and memory) and techniques to facilitate their expression. However, it does not specify how to use the movement component to achieve functional outcomes. Therapists place the child in reflex-inhibiting positions. Despite the popularity of this therapeutic approach, numerous studies34 and an American Academy of Cerebral Palsy and Developmental Medicine evidence report35 conclude that there are no discernible effects from traditional NDT. Patterning. Patterning has been a treatment option for children with motor impairments for more than 40 years. It involves a series of exercises designed to improve neurologic organization and is based on the premise that failure to complete properly any state of neurologic organization adversely affects all subsequent states. Advocates of patterning assert that the best way to treat a damaged nervous system is to “regress to more primitive modes of function and to practice them.”36 Patterning demands that the child’s head and extremities be manipulated in patterns presumably simulating prenatal and postnatal movements of typically developing children, by several persons over many hours during the day. Current information does not support the claims that this treatment is efficacious, and the Committee on Children with Disabilities of the American Academy of Pediatrics, states that its use is unwarranted.36 Conductive Education. Conductive education, developed at the Peto Institute in Budapest, is another motor intervention without clear benefit to children with cerebral palsy. It is defined as education rather than therapy. Conductors are trained to educate the children in how to control their bodies and overcome limitations.37 With the emphasis on education, children with severe mental retardation are considered ineligible for this program. There is emphasis on

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breaking motor tasks down into simple steps, problemsolving skills, and the use of rhythm and music to facilitate learning. A comparison of the outcomes after the participation of 34 children with cerebral palsy, matched by age, motor impairment, and cognitive ability, in programs of conductive education or NDT found that children show similar progress in each setting.38 Hyperbaric Oxygen Therapy. Parents eagerly grasp at promising treatment interventions for their children. For example, within the past few years, hyperbaric oxygen therapy has been introduced as a means to restore neurologic function in children with cerebral palsy. This treatment approach is based on the assumption that irreversibly damaged brain tissue is surrounded by viable but inactive tissue.39 Advocates claim that “flooding” these areas with an increased partial pressure of oxygen restores activity and function.40 Treatments are delivered in hyperbaric oxygen tanks, where the child breathes 100% oxygen at 1.5 to 2 atmospheres of pressure for 60 to 90 minutes.41 There are reports that hyperbaric oxygen therapy reduces hypertonicity and seizure frequency and enhances cognitive and communication skills. However, most of these findings are based on highly subjective clinical observations.39,42 A double-blind, randomized clinical trial showed no significant improvement in children with cerebral palsy after 40 daily treatments with 100% oxygen at a pressure of 1.75 atmospheres absolute for 60 minutes.43 There is no evidence substantiating the role of hyperbaric oxygen therapy in treatment of children with cerebral palsy. Moreover, treatments are associated with risks of oxygen toxicity, pneumothorax, barotrauma, and reversible myopia. Nonetheless, families have invested financial, personal, and emotional resources with the hope that this treatment might benefit their child. Forced Use or Constraint-Induced Movement Therapy. Forced use or constraint-induced movement therapy can be an effective technique to increase the use of the affected arm in a child with hemiplegic cerebral palsy. It entails restraining the stronger arm to force the weaker extremity to become more functional. For children who become frustrated and intolerant of prolonged immobilization of their dominant upper extremity, shorter intervals of decreased restraint may be effective. This can be accomplished with bivalved casts, splints, or slings, according to the needs of the child. Increased use and improved function of the weaker upper extremity have been observed in chil-

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dren with hemiplegic cerebral palsy.44,45 Large, controlled investigations of the efficacy of constraintinduced therapy in children with cerebral palsy are warranted. Practical Applications. In practice, most therapists offer a combination of therapeutic interventions for the child with cerebral palsy. Neurophysiologic and neurodevelopmental treatment can be combined into the neurodevelopmental approach, which includes methods to facilitate antigravity movement patterns and the sequential development of motor control. Repetition as a means of learning is stressed.46 Biomechanical, neurophysiologic, developmental, and sensory integration approaches to motor therapy are applied with the specific needs of the individual child in mind. Therapy prescriptions should include the child’s diagnosis, functional goals, and precautions as well as the type, frequency, and duration of therapy. The basic goals of motor therapies for children with cerebral palsy are to facilitate normal motor development and function, to prevent secondary deformities and/or disabilities, and to improve functional outcomes, community integration, and family adjustment.34 Thus, emphasis has shifted from a rather strict focus on impairments to a broader focus on the function of the child, at the level of the individual, the family, and the society.47 Research Directions. Recent studies have not demonstrated consistent treatment effects of developmental therapy in children with cerebral palsy, but the quantity and scope of the research is insufficient to make global statements about treatment outcomes. Treatments are neither standardized nor delivered in discrete amounts under controlled conditions. Research efforts are further limited by the inability to objectively measure the impact of the intervention on functional daily activities and the translation of motor skills into coordinated motor functions.35 The majority of studies are based on small numbers of subjects and on short-term interventions provided in poorly controlled conditions.48 Research trials placing children with cerebral palsy into treatment and no-treatment groups raise ethical issues. The influence of treatment timing, intensity, parent involvement, treatment combinations, and the profile of the children’s associated disabilities must be considered in research design.47

Interdisciplinary Care The concept of interdisciplinary care has emerged as professionals recognize that no one person or disci-

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pline has the skills to address the multifaceted nature of children with developmental disabilities.27 Interdisciplinary care has evolved more recently to focus on the provision of family-centered care. Interdisciplinary care should not be confused with multidisciplinary care, in which several professionals work independently of each other on aspects of care within their own areas of expertise. An interdisciplinary team is collaborative and problem-oriented rather than discipline-oriented.27 Thus, although the care is time- and labor-intensive, it is the recommended model of care for children with cerebral palsy. The World Health Organization (WHO) model of assessing health status in adults with musculoskeletal disabilities was proposed by Mati in 1980.49 This has been expanded by the National Center for Medical Rehabilitation Research (NCMRR) in 1995.50 The model describes levels of dysfunction and includes pathophysiology (cellular level), impairment (organ level), disability, or functional limitations (individual level) and handicap or societal limitations (societal level). In the opinion of the authors, if the interdisciplinary team approaches the functional assessment of the child with cerebral palsy in terms of this model, a comprehensive plan of disease management will logically follow. An additional role of the interdisciplinary team is to guide the families in interpreting the scientific evidence currently available and to avoid the roller coaster ride of unsubstantiated interventions. The key participants on any interdisciplinary treatment team are the child and family as well as physical, occupational, and speech therapists. Physical therapists focus on gross motor skills, including sitting, standing, walking, wheelchair mobility, transfers, and community mobility. They order mobility equipment and assistive devices and often perform inhibitive and serial castings.51 Occupational therapists address the visual and fine motor skills that enable coordinated functions of activities of daily living such as dressing, grooming, toileting, eating, bathing, and writing. They assess a child’s school readiness and facilitate the development of compensatory strategies, often through the application of environmental control units and adaptive toys.29 Speech language pathologists focus on the communication and cognitive abilities of the child and recommend augmentative communication devices when indicated. Interdisciplinary teams also include pediatricians and subspecialists, social workers, case managers, nutritionists, educators, and others, as indicated by the individual needs of the

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children and their families. Although each child may not be in need of all members of the team all of the time, the interdisciplinary setting allows for the most effective care delivery system.15

Early Intervention It is accepted that every child who is developmentally at risk should be provided with early intervention services. Do we know that early intervention services are beneficial? We know that early intervention helps socially disadvantaged children, improves family adaptation, and functioning and helps children reach their developmental potential. We know that the most effective early intervention programs are those that begin early and are highly structured and familycentered. We know that children who receive early intervention programming need fewer special education services later in life.52 Early institution of physical, occupational, and speech therapies fosters a more normal neuromotor developmental sequence, maximizes range of motion, and teaches the patient and family how to implement a home therapeutic program.15 Young children with cerebral palsy are often limited in their ability to interact socially, initiate communication, and explore and manipulate their environments. Cognitive and communication abilities can be masked by motor impairments. Less sensory, social, and language input may be offered when compared with typically developing children. It has been demonstrated that repetition facilitates the acquisition and expression of motor skills and that learning, social interaction, and experience are integrally involved in the process of motor development in all children, regardless of abilities.34 Thus, early provision of therapeutic activities is believed to be of paramount importance. Federally mandated special education and related services for children with disabilities originated in 1975 (Public Law 94 –142).47 More recently, the Individuals with Disabilities Education Act (IDEA, Public Law 101– 476) and the Americans with Disabilities Act (ADA, PL 101–336) have provided children and adults with developmental motor disabilities opportunities to participate more fully in mainstream society.21 The law requires that “states develop, implement, and fund statewide comprehensive, coordinated, interdisciplinary programs for infants and toddlers with disabilities and their families.”52

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Early intervention programs, administered by Maternal and Child Health through the Department of Health and Welfare, specifically address the developmental needs of children from birth through 36 months.52 The referral for assessment can be made by any concerned individual. To determine eligibility, an interdisciplinary team evaluates the infant or toddler across five domains, including physical, cognitive, communication, social/emotional, and adaptive development. In general, children scoring 1.5 to 2.0 standard deviations below the mean on one or more of the developmental domains qualify for services. A plan for services, or an Individualized Family Service Plan (IFSP), is designed for each child and family and recognizes that the parents are the expert decisionmakers and care providers for their children. The intervention must be provided in the most natural or least restrictive environment, which is often the home. State-to-state variability exists in the fraction of children enrolled in early intervention programs. Local availability of resources, program enrollment criteria, and local traditions of care can influence enrollment.27 Utilization rates range from 1% to 72%, further reflecting the lack of consensus about how to implement developmental therapies.21

Family Issues Disclosing the diagnosis of cerebral palsy to parents is a challenging task for the clinician. Interviews with parents of children with cerebral palsy demonstrated that the initial dissatisfaction with disclosure was related to the child’s degree of prematurity, the severity of disability, and when the disclosure was made relatively late. Dissatisfaction was also related to later self-reported levels of depression.53 It is recommended that the physician set aside sufficient time, meet with more than one care giver (without the child present), engage in dialogue rather than monologue, and avoid a gloomy attitude about the diagnosis and prognosis.27 A follow-up meeting or phone call provides an opportunity to clarify the information and answer questions from parents who might have been overwhelmed during the initial discussion. The reaction of the family to hearing that their child has cerebral palsy may depend on religious and cultural backgrounds, perceptions, and attitudes regarding disabilities, health and healing techniques, motivation, and the tolerance to allow others to assist with care.27 Some families are greatly relieved to finally get answers and help for their child, whereas

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others may feel anger toward the professionals who reassured them that their child would “grow out of it.”27 Families need time to grieve the loss of their anticipated “normal” child and to move through the stages of grief as defined by Kubler-Ross, including denial, depression, anger and guilt, and bargaining and acceptance.27 Over time, most families can cope effectively with their child’s disability, and some families have reported increased understanding and compassion within the family and others, a more enriched and meaningful life.27

Clinical Scenario: The Preschooler (3 to 5 Years) A father brings his 4-year-old daughter with spastic diplegic cerebral palsy to your office. He is concerned about his wife’s level of exhaustion, the family finances, and the development of his child, who sleeps poorly, is chronically constipated, and has not yet completed potty training. On examination, you note that her weight is below the 3rd percentile for age and height below the 25th.

Feeding, Nutrition, and Growth Oral motor impairments have been noted in 90% of children with cerebral palsy54 and gastrointestinal issues in 80% to 90%.55 Approximately one third of children with cerebral palsy are undernourished, and many show the consequences of malnutrition. Children with severe cerebral palsy typically have linear growth reduced to below the 3rd percentile, with progressively delayed growth with age.56 Milder cerebral palsy is characterized by lesser degrees of growth delays. The reduced lean muscle mass of children with cerebral palsy is related to reduced linear growth, muscle mass depletion, and disuse atrophy. Total energy expenditure is decreased as the result of lack of physical activity.57 Although growth delays appear to be multifactorial in origin, the leading cause appears to be poor nutrition secondary to oral dysphagia. Oral motor skills are often affected in children with cerebral palsy, especially those with the spastic quadriplegic subtype. Dysphagia can make the feeding experience unpleasant for both the child and the care giver, and poor oral motor skills can contribute to prolonged feeding times. Gastroesophageal reflux reduces the number of calories available for growth, increases the risk for aspi-

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ration, and can be a source of pain and an additional reason for food refusals in the difficult-to-feed child. It is observed that children with cerebral palsy are offered about 10% less food and consume only about one half as much as control children. Caregivers do not verbalize to their young children during mealtimes and describe the feeding experience as unpleasant or difficult.54 Nonnutritional factors also contribute to the occurrence of growth delays in children with cerebral palsy. Children with hemiplegic cerebral palsy, each serving as his or her own control, demonstrate smaller and shorter limbs and thinner skinfold thicknesses on the involved side.58 Limited weight-bearing abilities and muscle imbalances establish abnormal stresses on immature bones, which may contribute to growth delays. Early nasogastric or gastrostomy tube feedings for children with cerebral palsy lead to increased weight for length relations, improved immune competence, enhanced overall alertness and responsiveness, and greater family satisfaction.58 Early, persistent and severe feeding difficulties in infancy are predictive of subsequent poor growth, feeding, and developmental outcomes and can therefore be used to identify children with cerebral palsy who might benefit from gastrostomy tube feedings.59 Nasogastric tubes can be used for short-term nutritional support in children with cerebral palsy, but typically long-term intervention is indicated. Nasogastric tubes on a long-term basis are associated with unaesthetic appearance, nasal discomfort, irritation or penetration of the larynx, recurrent pulmonary aspiration, and tube blockage or displacement. They may contribute to oral aversion and limit the development of oral feeding skills.56 Surgically or percutaneously placed gastrostomy tubes lead to greater weight gain when compared with nasogastric tubes and are preferred by patients, parents, and nursing staff.56 A survey of parents of children with cerebral palsy who received gastrostomy tube feeding during an 8-year interval revealed that 90% were pleased with the effect of tube feeding on the family.60 It is essential that gastroesophageal reflux be managed effectively before the establishment of enteral feedings because rapid nutritional rehabilitation is associated with increased morbidity and mortality rates secondary to gastroesophageal reflux, aspiration pneumonia, peritonitis, or refeeding syndrome.56 Medical therapies are focused on optimizing control of intragastric pH and increasing motility of the gastro-

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intestinal tract. Baclofen, commonly used for spasticity control, has been shown to reduce reflux through inhibition of transient lower esophageal sphincter relaxation.61 Fundoplication is often indicated at the time of gastrostomy tube placement. However, postoperative complication rates in children with severe erosive esophagitis and cerebral palsy are 26%, compared with 12% in neurologically intact children.55 More recently, gastrojejunal tubes have been proposed as a less invasive option.62 Infrequently, one encounters the child with cerebral palsy who is either overweight or obese. In addition to the widely recognized risks of childhood obesity, excess adiposity can prevent or severely limit the child’s potential for independent mobility.

Toileting Issues Bladder Function. Children with cerebral palsy are at risk for several problems related to the urinary tract, including incontinence, urgency, frequency, retention, and infections.27 Spasticity and hyperreflexia of the skeletal muscles can be accompanied by spasticity of the detrusor, leading to small frequent voids and a contracted, low-capacity bladder. Upper motor neuron urodynamic abnormalities have been demonstrated in 87% of the children with cerebral palsy referred to urologists for symptoms of neurogenic bladder dysfunction. Nevertheless, structural abnormalities of the genitourinary tract are infrequent, and routine evaluation of asymptomatic children with cerebral palsy is not recommended (Brodak et al, J Urology, 1994). Hypertonicity of the pelvic floor has been shown to be a contributing factor to voiding difficulties in adults with cerebral palsy.63 Primary incontinence has been reported in 23.5% of children and adolescents with cerebral palsy between the ages of 4 and 18 years. Urinary incontinence is associated with low intellectual function and quadriplegia, with only 38% becoming continent by 6 years of age.64 The attainment of urinary continence involves maturation of the urinary tract, the autonomic nervous system, and the frontal and parietal lobes. It requires the child’s awareness of bladder fullness and the abilities to inhibit bladder reflex contractions and to void volitionally.64 The child’s ability to communicate toileting needs and to promptly get to the bathroom and manage clothing also influences the attainment of continence. Adapted toilet seats, handrails, or clothing modifications can increase toileting successes for children with cerebral palsy.

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Bowel Issues. Constipation is a common problem among children with cerebral palsy. It contributes to feeding problems, loss of appetite, growth impairments, and irritability. When severe and chronic, permanent intestinal dysmotility can result, and in extreme instances, bowel perforation can occur.27 A bowel management program is initiated with a cleanout phase, typically requiring a combination of laxatives to evacuate the upper intestinal tract and enemas or suppositories to clear the lower tract. Afterward, a schedule of softening agents such as polyethylene glycol, docusate sodium, or senna are coupled with a diet generous in fiber and fluid to produce regular and soft bowel movements. It is recommended that bowel programs be performed 30 minutes after a meal, taking advantage of the gastro-colic reflex, and may be further stimulated with bisacodyl or glycerin suppositories.27 With effective bowel management programs, some children can attain bowel continence through regularly timed evacuations.

aims of orthotic interventions as the prevention and/or correction of deformities, provision of support, facilitation of skill development, and improvement of gait efficiency.65 Orthoses are named by the joints they are designed to support. The most common orthoses used by children with cerebral palsy are ankle-foot orthoses (AFOs), designed to hold the heel and forefoot in optimal biomechanical position. AFOs vary in the degree of support they provide and amount of motion they allow. Hand and wrist orthoses can be used during daytime hours to enhance function by supporting weak joints or inhibiting abnormal patterns of movement. Orthoses designed to prevent or correct deformities may be worn overnight, according to the child’s tolerance.27 Knee immobilizers are worn overnight to position the knee in extension and to passively stretch the hamstrings. Typically, orthoses are refabricated yearly, due to growth and wear. Like all therapeutic interventions, the selection of orthoses must be based on specific and individualized goals.

Adaptive Equipment

Sleep Disturbances

Standers are devices that support the nonambulatory child with cerebral palsy in an upright position and facilitate weight bearing through the lower extremities. They improve musculoskeletal alignment, promote bone mineralization, and decrease the occurrence or severity of lower-extremity contractures in children with cerebral palsy.27 Mobility devices include walkers, crutches, and manual and power wheelchairs. Posterior walkers are preferred over anterior walkers for children with cerebral palsy because they promote trunk extension and limit hip flexion.27 Children who are able to walk with assistance during their early years may find that the time and energy costs of ambulation become excessive due to increased body mass and the development of orthopedic complications. As adolescents, they may naturally transition from ambulating with adaptive equipment to independent, energy- and time-efficient community mobility in manual wheelchairs. They should be encouraged to continue household ambulation. Wheelchairs can allow the children to keep up with peers in social, educational, and recreational activities and to develop independence.27 Power wheelchairs are appropriate for children who lack the strength, coordination, or endurance to propel a manual chair but demonstrate the cognitive skills necessary for safe navigation. A consensus statement from the International Society for Prosthetics and Orthotics identifies the primary

Children with cerebral palsy may struggle to initiate or maintain sleep.66 Abnormal sleep EEG patterns, including the absence of REM sleep, abnormalities of the sleep spindles, and a high incidence of awakenings after sleep onset, have been reported in 50% of persons with cerebral palsy.67 Central and obstructive sleep apnea occurs at a greater frequency in individuals with cerebral palsy and can further contribute to sleep disorders. Medications that improve the sleep-wake cycle in children with spastic cerebral palsy may also decrease hypertonicity. This appears to result from the reversal of sleep deprivation rather than the direct pharmacologic action of the medication.68 Intrathecal baclofen therapy for spasticity has been shown additionally to facilitate sleep by decreasing apneic events and reducing the frequency of disturbing involuntary leg movements.69 Melatonin and clonidine have both been shown to positively affect the sleep-wake cycle of children with multiple disabilities. Medications should be slowly titrated to avoid morning drowsiness, and ongoing indications should be regularly reassessed.70,71

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Drooling It is estimated that some degree of drooling occurs in 25% to 35% of all persons with cerebral palsy, secondary to failure of lip and jaw closure, tongue

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thrusting, and dysphagia.72 It is not, however, related to excess production of saliva. Drooling can lead to aspiration of oral secretions, discomfort, skin maceration, compromised hygiene, impairments of articulation, transmission of infection, and social isolation.72 Current management strategies for excessive drooling in children with cerebral palsy yield disappointing results. A limited number of children benefit from behavior modification programs that use auditory alarms.73 Anticholinergic medications decrease salivary flow by blocking parasympathetic innervation to the salivary glands. Glycopyrrolate is most often prescribed, although it has side effects of irritability (10%) and constipation (7.5%).30 Less frequently, treatment with glycopyrrolate can produce blurred vision, urinary retention, and sedation. Scopolamine is another anticholinergic agent that has been used to reduce sialorrhea.74 It is available in a transdermal delivery patch but can lead to heat sensitivity secondary to decreased perspiration, increased irritability, and in some instances, reversible toxic psychosis. Surgical denervation of the salivary glands (tympanic neurectomies) provides short-term relief, although drooling returns to baseline over time. Ligation and/or rerouting of salivary ducts may lead to increased aspiration events, and removal of salivary glands is associated with discomfort and accelerated tooth decay.30 A preliminary study suggests that botulinum toxin A injected into the parotid and submandibular salivary glands may be an effective management strategy for excessive drooling.75,76 Currently, management strategies remain suboptimal.

establish specific and measurable education goals.27 The school district must provide essential services to ensure educational success for each student.77 Reevaluations are required minimally every 3 years but are usually held annually. Once the student reaches 14 years of age, the IEP must also address transitional programs such as vocational education, with yearly updates. A statement of needed transition services and interagency responsibility is mandated by age 16 years.27 The Rehabilitation Act of 1973 is a comprehensive federal law that provides for state vocational rehabilitation services, commissions for the blind, independent living centers, a National Council on Disability, and a client assistance program. Section 504 of the Rehabilitation Act specifically prohibits discrimination on the basis of disability. It requires that eligible students receive educational services, reasonable accommodations, and related aids to ensure that their needs are met just as the needs of nondisabled students are met. Individuals who have a physical or mental impairment that limits a major life activity are eligible. Life activities include walking, seeing, hearing, speaking, breathing, learning, working, caring for oneself, and performing manual tasks. The Federal Motor Vehicle Safety Standards (FMVSS) address safe transportation for children with disabilities on special education school buses.78 Transportation goals are specified in a child’s IEP.79 Pediatricians can advocate for children by ensuring that parents are aware of the safety standards and that local school systems are compliant with requirements.

Rights of the Student With Cerebral Palsy

Family

Each child with cerebral palsy deserves a smooth transition from early intervention services to the preschool setting. Children ages 3 to 5 years may be eligible for developmental preschool programs, where group rather than individual therapies are rendered. The Individuals with Disabilities Education Act (IDEA) is a federal education law that provides grants to assist states in providing special education services to children with disabilities. This Act grew out of the 1975 Education for All Handicapped Children Act (PL 94 –142), which mandates a free, appropriate public education for all individuals with disabilities.27 Children ages 3 through 21 years are eligible for services through IDEA if they have a disability and need special education. An Individual Education Program (IEP) is developed by the team and parents to

Mothers of children with cerebral palsy between the ages of 1 and 5 years participated in a series of interviews. They reported that strong extended family relationships are invaluable, that day-to-day care giving is difficult, that increasing their knowledge of cerebral palsy improves the children’s quality of life, and that the family financial status is affected.80 The care needs of children with severe disabilities are significantly greater than those of typically developing children and do not decrease with advancing age. Often, mothers are unable to work outside of the home and therefore family income is frequently less than peer families of nondisabled children.81 The coping ability of the family is influenced by many factors, including marital status, support systems, and previous life experiences. Burnout can result from the daily

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stresses of parenting a child with functional limitations and leads to care giver fatigue, emotional exhaustion, frustration, and generalized life dissatisfaction.27 The coping styles of the parents influence that of the siblings. Siblings may worry that the disability is contagious and that they might become affected, or that in some way, they caused their sibling’s disability. Some siblings engage in attention-seeking behaviors and others withdraw to avoid further stressing the parents. Siblings do best when the disability is explained honestly and when they are not expected to perform excessive child care responsibilities.82 Internet resources can help children with cerebral palsy and their families identify valuable informational, support, and community services (Appendix).

Clinical Scenario: Childhood Years (6 to 12 Years) An 8-year-old boy with spastic quadriplegic cerebral palsy who has received treatment with high oral doses of baclofen and repeated botulinum toxin injections has hip pain and decreased range of motion. He is sent to your office by the school for concerns of sitting intolerance and crying during diaper changes.

Spasticity and Its Management Definitions. Spasticity, the most common motor abnormality associated with cerebral palsy, is defined as increased velocity-dependent resistance to movement.10,83,84 It is characterized by both positive and negative symptoms. The positive symptoms include hypertonicity, clonus, and hyperreflexia and are generally amenable to treatment interventions.85 Decreased motor planning, loss of selective motor control, weakness, and poor endurance are the negative symptoms of spasticity and are difficult to modify.86 In general, there is an inverse relationship between the level of spasticity and degree of voluntary motor control.87 Spasticity in a child with cerebral palsy does not universally demand treatment, and its impact on function must be assessed. Children may rely on lowerextremity extensor tone to assist with transfers and ambulation. Spasticity management therefore must be goal-specific, such as to assist with mobility, reduce or prevent contractures, improve positioning and hygiene, and provide comfort. Each member of the child’s interdisciplinary team, including the child and

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parents, participates in treatment planning and serial evaluations.84 The physician alone may not appreciate the full spectrum of the child’s abilities and limitations in a single office visit. Any noxious stimulus, such as pain, infection, excitement, temperature changes, fatigue, or stress, can aggravate hypertonia and thereby affect examination findings.86 Similarly, when a child with stable spasticity has an acute change in motor control or function, the physician needs to look for underlying causes. Constipation, gastroesophageal reflux disease, urinary tract infections, or hip dislocations may be the triggering factors, and treatment should be directed accordingly. The child with spastic cerebral palsy is best cared for with an individualized treatment plan that provides a combination of interventions rather than monotherapy. Physical interventions such as range-of-motion exercises, the use of orthotic and adaptive devices, and strength and mobility training activities are the foundation of all therapeutic programs. Local and systemic medications and orthopedic and neurosurgical procedures are needed periodically at different developmental stages and in different combinations over the years. Treatment Options for the Management of Spastic Cerebral Palsy. Physical and occupational therapies include range of motion exercises; strength, coordination, and functional activities; orthotic devices and adaptive equipment; and inhibitive and serial casting. Intramuscular injections and serial casting include botulinum toxin and phenol. Systemic medications include baclofen, benzodiazepines, dantrolene sodium, tizanidine, and clonidine. Orthopedic surgeries include tendon lengthening, transfers, and osteotomies. Neurosurgical surgeries include selective dorsal rhizotomy and intrathecal baclofen therapy. Local Spasticity Interventions. Spasticity in children with cerebral palsy can be focal, generalized, or unequally distributed in the extremities. In such instances, botulinum toxin can be a highly effective tool.88 Botulinum toxin is an injectable, purified protein derivative that blocks the release of acetycholine at the neuromuscular junction. It is injected into the muscle belly through surface anatomic localization and spreads by diffusion. This quick office-based procedure is generally well tolerated. Botulinum toxin has an onset of action of 3 to 10 days and an average therapeutic duration of 3 to 6 months.87 With an optimal outpatient and home program, longer benefits from the injections can occur. Side effects are infrequent and may include transient swelling, flu-like

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symptoms, and local muscle weakness. Dosages range from 10 to 20 units per kilogram or 1 to 2 units per kilogram per muscle, depending on the muscle size, the degree of spasticity, and the individualized functional goals. Injections of botulinum toxin can be directed into gastrocnemius muscles to reduce toe-walking and plantar flexion contractures and are often followed by serial casting and orthotic interventions. Hip adductors can be targeted to control scissoring and to reduce the risk or slow the progression of hip subluxations or dislocations. The reduction of hamstring spasticity can improve posture, balance, and comfort in sitting and reduce lower extremity crouching during gait. Botulinum toxin injections can be used to delay orthopedic surgeries until timing is ideal. Upper extremity injections can also be beneficial. Careful and appropriate selection of sites for injection, dosages, and associated therapies is essential to ensure optimal outcomes. The effectiveness of botulinum toxin in the management of spastic cerebral palsy has been verified by objective measurements in multiple prospective studies.88 It is imperative to have an interdisciplinary, goaldirected treatment plan in place before the administration of botulinum toxin. To optimize the duration and quality of outcomes, plans for serial casting and bracing, modifications in the frequency or type of therapy services, and the details of home therapy programs should be made with the child and family in advance. Positioning devices and orthoses worn during sleep can provide much-needed prolonged stretch during times when muscles are maximally relaxed. If the child’s sleep is disrupted, then alternative schedules should be established.89 Phenol is a neurolytic agent used in the treatment of focal muscle spasticity in selected children with cerebral palsy. It is administered in concentrations of 2% to 5%, noting that concentrations ⬎5% can lead to protein coagulation and necrosis. The onset of action is immediate, and duration of effect is from 4 to 12 months.87 Because phenol injections require electromyographic guidance to localize motor points and involve more painful and lengthy procedures, anesthesia is indicated. Moreover, phenol injections have been reported to cause dysesthesias and neuropathic pain syndromes in approximately 10% of patients.90 If phenol is injected intravascularly, seizures, cardiac arrhythmias, and hypotension can occur.87 Systemic Spasmolytic Medications. Systemic treatment options for the control of spasticity in children

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with cerebral palsy include baclofen, diazepam, dantrolene, and tizanidine, alone or in combinations.86,87 Clonazepam, gabapentin, and clonidine are less frequently used. Baclofen is the most commonly used antispasticity medication in children with cerebral palsy. Spasticity results from an inadequate release of gamma-aminobutyric acid (GABA), an inhibitory neurotransmitter in the central nervous system. Baclofen is a structural analog of GABA and therefore exerts its action by enhancing presynaptic inhibition.91 In high doses, it can also depress the excitability of postsynaptic neurons.92 Baclofen has a half-life of 3.5 hours and is rapidly absorbed after oral administration. Side effects of baclofen include fatigue, irritability, hypotension, increased drooling, impairments of memory and attention, and lowered seizure threshold. These negative effects can be minimized by slow upward titration of the medication. Abrupt withdrawal of baclofen initially results in rebound spasticity and irritability and subsequently fever, hallucinations, and status epilepticus.83 Benzodiazepines, including diazepam, clonazepam, and clorazepate, can also be useful in the management of spasticity in children with cerebral palsy. Like baclofen, these drugs act to increase presynaptic neuronal inhibition through GABA pathways.93 Sedation is the most common side effect with the benzodiazepines and therefore may be the preferred medication for young children with spastic cerebral palsy and sleep disturbances, anxiety, or irritability. Physiologic tolerance can occur, requiring dosage increases to maintain therapeutic results. The half-life of diazepam is 20 to 80 hours, clonazepam is 18 to 28 hours, and clorazepate is 106 hours.83 Dantrolene sodium is infrequently selected to control spasticity in children with cerebral palsy because its pediatric safety profile is unclear. Dantrolene exerts its action at the muscular level by inhibiting the release of calcium from sarcoplasmic reticulum and thereby uncoupling excitation and contraction.92 Muscle weakness is the major side effect. Fatigue may occur during treatment with dantrolene but is usually less than with baclofen or the benzodiazepines.87,94 Dantrolene has a hepatotoxicity rate of 1.8% in adults. The effect on a child’s liver is uncertain, although there have been no reports of hepatotoxicity in children younger than 16 years of age.94 Liver function tests should be monitored before starting therapy and periodically thereafter.87

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Tizanidine and clonidine are alpha-2 adrenergic agonists that reduce spasticity by hyperpolarizing motoneurons and decreasing the release of excitatory amino acids. This leads to an overall reduction in motoneuron excitability. Side effects may include nausea, vomiting, hypotension, sedation, depression, and hepatotoxicity.83 Clonidine is available for transdermal and oral administration. The half-life of oral clonidine is 5 to 19 hours, and that of tizanidine is 2.5 hours. Orthopedic Procedures. Immature bones grow in the direction of the forces placed upon them. The abnormal muscle tone and imbalances characteristic of spastic cerebral palsy can lead to progressive joint contractures, shortened muscles, and tensional deformities of the hip and foot.87 Chronic shortening of spastic muscles leads to contractures that often require surgical intervention.15 Soft tissue orthopedic procedures should be considered whenever an anatomic structure is at risk as a consequence of the spasticity, with the primary goal of preventing serious bony deformities.95 Optimal spasticity control needs to be established before and after soft tissue procedures to ensure favorable long-term outcomes. Children with spastic cerebral palsy often undergo lengthening procedures of the hip flexors, adductors, hamstrings, and triceps surae. Obturator neurectomies may accompany adductor releases to reduce abnormal muscle activity.51 Historically, orthopedic surgeries were scheduled individually, addressing soft tissue releases and bony deformities one at a time. It is now recognized that simultaneous multiple surgeries allow the child and family to avoid recurrent hospitalizations and extended periods of casting and rehabilitation.15,27 Russman suggests that one consider the low back, hip, knee, and ankle as four weights on the corners of a suspended balance board. Unless weight is subtracted or added evenly at all four corners, the board will tip. The muscle imbalance must be precisely defined before surgery when multiple procedures are planned.1 The timing of soft tissue surgeries in children with cerebral palsy is critical. Ideally, procedures are planned after the child has developed a mature gait pattern, typically by 5 years of age. In general, surgeries are not recommended before 4 years of age to avoid overcorrection. Rapid rates of growth, postural maturation, and physiologic ligamentous tightening during the first few years of life may alter the surgical plans for a child with spastic cerebral palsy.51 A child’s muscles will double their birth length by 4

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years and double again by adulthood.27 Thus, muscle lengthening performed before 4 years of age often needs to be repeated in later years.27 Lengthened muscle is also weakened muscle, and postoperative rehabilitation is essential. Selective Dorsal Rhizotomy. Selective dorsal rhizotomy is a neurosurgical procedure that reduces the lower extremity spasticity of cerebral palsy. The procedure involves intraoperative electromyographic monitoring to identify the sensory rootlets from L2 to S2, which, when stimulated, result in abnormal motor responses. Approximately 50% of the stimulated rootlets are cut.96 Laminotomies, with replacement of the bony spinal arches, are now preferred over multilevel laminectomies, resulting in lower rates of spinal complications such as spondylolysis, spondylolisthesis, and excessive lumbar lordosis.97 The ideal candidate for selective dorsal rhizotomy is the cooperative, motivated child with spastic diplegic cerebral palsy who demonstrates good strength, balance, range of motion, and isolated muscle control86 but is functionally limited by spasticity.97 Although selective dorsal rhizotomies effectively reduce lower extremity spasticity, there may be postoperative indications for additional procedures. For example, shortened muscles and contracted joints may need orthopedic surgeries to bring the child to his or her full functional potential.87 The participation of the child and parents in postoperative rehabilitation programs is essential. The selective dorsal rhizotomy has been shown to reduce lower extremity spasticity and thereby improve joint range of motion, gait, and gross motor and functional abilities. It has also been reported to decrease the incidence of hip dislocation.96 In a metaanalysis of three randomized, controlled trials, children with cerebral palsy who underwent selective dorsal rhizotomies followed by physical therapy programs had significantly reduced spasticity scores and higher functional measure scores when compared with children who received physical therapy alone. Moreover, those children who had larger percentages of dorsal roots transected demonstrated significantly more improvement on the Gross Motor Functional Measure (GMFM).98 Even though selective dorsal rhizotomy primarily affects lower extremity spasticity, there are reports of concommitant reductions in upper extremity spasticity.93 Additionally, improvements in attention and cognitive functioning after surgery are

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reported, over and above the general improvement in temperament or physical comfort.99 Intrathecal Baclofen Therapy. Baclofen has been used systemically to reduce spasticity since the 1970s. Intrathecal baclofen was introduced in the 1980s, initially for the treatment of spasticity of spinal origin. It is now widely used for adult and pediatric spasticity of cerebral origin. A programmable, refillable intrathecal baclofen pump is surgically implanted into a subcutaneous abdominal pocket and is connected to a catheter system, which delivers a continuous infusion of baclofen into the spinal canal. Baclofen, when taken orally, crosses the blood-brain barrier poorly. With direct delivery of medication to the site of action, intrathecal baclofen doses are generally one hundredth of oral baclofen doses. Children with spastic cerebral palsy, with or without dystonia, who have spasticity refractory to treatment with oral medications or are intolerant of the side effects, may be candidates for intrathecal baclofen therapy. The child participates in a screening trial, during which a bolus of baclofen is injected through lumbar puncture, followed by a period of observation to assess response.100 If the screening dose successfully lowers muscle tone, decreases the frequency and severity of spasms, and/or decreases pain, the child then undergoes implantation of the pump and catheter delivery system. Pumps are refilled transcutaneously approximately every 3 months. The literature consistently reports significant shortterm and long-term reductions in upper- and lowerextremity spasticity with intrathecal baclofen therapy.91 Upper-extremity spasticity is better controlled by higher concentrations of baclofen and higher catheter placements. Additional benefits of intrathecal baclofen therapy in children with cerebral palsy include reduced pain, increased endurance, heightened alertness, improved nutritional status,91 and increased self-reported quality of life.101 Children receiving intrathecal baclofen therapy may have complications related to mechanical failures or the medication itself. Mechanical complications include cerebrospinal fluid leaks, pocket seromas, and pump failures, although catheter kinks, disconnections, fractures, and dislodgment are most common. The implanted system can become infected, leading in some instances to meningitis and requiring explanation of the system.102 Reversible overdoses of intrathecal baclofen, typically caused by programming errors, lead initially to somnolence, hypotonia, and

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respiratory depression and progress to loss of consciousness and respiratory failure.97 Withdrawal after the abrupt cessation of intrathecal delivery can be life-threatening, with severe hypertonicity progressing to rigidity, seizures, hyperthermia, rhabdomyolysis, and multiorgan failure.103,104 Early recognition and intervention are essential.

Hips The overall prevalence of hip subluxation or dislocation in children with cerebral palsy is between 22% and 45%.27 Nearly one in four children with quadriplegic cerebral palsy has paralytic hip dislocation.105 Hip subluxation or dislocation has been demonstrated in 7% of those who ambulate independently and in 60% of those who sit dependently.106 Hips may dislocate as early as 18 months, most frequently in nonambulatory children with spastic types of cerebral palsy.27 Like most of the orthopedic problems encountered in children with cerebral palsy, hip deformities result from abnormal muscle tone and activity on immature bones15 and progress with growth. When spastic hip adductors and flexors overpower the abductors and extensors,106 coxa valga, excessive femoral anteversion, and acetabular dysplasia follow.15 Persistent primitive reflexes and inadequate weightbearing activities further contribute to the development of hip dislocation in children with cerebral palsy.107 Hip subluxations and dislocations in children with cerebral palsy can be detected on physical examination, with the child placed supine on the examination table and hips flexed to 90 degrees. Limitations or asymmetries in hip abduction or thigh shortening are abnormal.27 Radiographs of the pelvis are indicated at least annually in all at-risk children with cerebral palsy. Subluxation of the hip refers to a partial displacement of the femoral head in the acetabulum and may progress to dislocation, in which the femoral head is completely displaced. Despite timely treatment with positioning techniques, botulinum injections, and soft tissue releases, subluxations may progress to dislocations. Surgical interventions for subluxed hips typically involve adductor tenotomies and myotomies, occasionally coupled with iliopsoas releases, lengthenings, or recessions when hip flexion contractures are also present.27 The primary goal of treatment is to prevent or treat pain, correct deformities, and improve range of motion.

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Operative interventions for hip dislocations in children with spastic cerebral palsy are much more extensive and often less successful than are those for subluxation. Procedures may include open reduction with femoral derotational osteotomies, pelvic osteotomies, and in extreme cases, resection arthroplasties, hip fusions, and total hip replacements.15 Early treatment is advisable: Degenerative arthritis and hip pain occurs in 50% of the children who receive no treatment108 and is the most common orthopedic complaint in adults with cerebral palsy.109 A comparison of treated and untreated chronically dislocated hips in nonambulatory persons with severe cerebral palsy demonstrated no significant differences in terms of level of pain, sitting ability, pelvic obliquity, scoliosis, difficulties with nursing care, pathologic fractures, and decubitus ulcers.110 This suggests that surgical treatment of already dislocated hips in individuals with severe cerebral palsy may not be helpful. Many late and chronic dislocations should be left alone, unless there is chronic pain.27 In all instances, the surgical interventions must be goal-directed and selected for the individual needs of the child and family.

Clinical Scenario: Adolescence A 15-year-old teenager with extrapyramidal quadriplegic cerebral palsy indicates that she has pain in her hips and lower back, especially when sitting in her wheelchair. In addition, the mother is concerned about her daughter’s limited peer interactions and wonders if her daughter might be depressed.

Becoming Adults Puberty is a tumultuous time for all adolescents, and teenagers with cerebral palsy are no exception. Adolescence is a time of self-evaluation and comparison with others, and teens with cerebral palsy may become increasingly aware of their physical differences and areas of competence.111 As a consequence of functional limitations, poor self-esteem and the attitudes and reactions of those around them, adolescents with disabilities often feel socially isolated.27 Because opportunities to develop social skills are often lacking, support and guidance are needed for the development of interests and activities in which the adolescent can be successful.27,112 The primary goal of habilitation during this developmental stage is the acquisition of skills to ensure a successful transition into adulthood.

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Older adolescents with cerebral palsy who participated in a small, qualitative study defined success as “being happy.” Being believed in (self-actualization), believing in yourself (achievement), and being accepted by others (affiliation) were identified as the three factors related to achieving success.111 The investigators found many more similarities than differences among teenagers with and without disabilities. When families of adolescents with and without cerebral palsy are compared, differences in family functioning, life satisfaction, and perceived social support are also found to be minimal.113 Adolescents with disabilities should be recognized as sexual human beings and encouraged to talk about their sexuality.27 They often have limited access to information about puberty and sexuality. In a survey of adolescents with cerebral palsy, 58% reported receiving some sex education in school, whereas only 12% discussed issues of sexuality in the home. There remains a tendency to infantilize adolescents with cerebral palsy.114 Parents may need encouragement to give their teenager the necessary freedom and support to become independent and take reasonable risks.27,115 The child with cerebral palsy may remain functionally dependent for his or her entire life. When expected life-cycle changes do not occur, such as graduations or marriages, family members may feel a reemergence of the sadness they had at the time of initial diagnosis.27 Support for the entire family is recommended during times of transition. Children with disabilities are 1.8 times more likely to be neglected, 1.6 times more likely to be physically abused, and 2.2 times more likely to be sexually abused than children without disabilities.116 They tend to be highly trusting of others and are more likely to be exploited and influenced by those who offer alcohol, drugs, or sex. Those who depend on caregivers for their physical needs may be accustomed to having their bodies touched regularly by adults.116 Children with disabilities can be perceived as easy victims of sexual abuse, because their intellectual limitations or communication impairments may prevent them from disclosing abuse.116 Data from 35 child protective service agencies across the country indicate that 14.1% of the children whose maltreatment is substantiated have one or more disabilities.116 Children with chronic illnesses or disabilities often place high emotional, physical, economic, and social demands on their families. Parents with limited social, community, and respite support may be at risk for maltreating

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children with disabilities because of feeling overwhelmed and unable to cope. The prevention and recognition of child abuse should be discussed openly with parents and caregivers. Children should be given information within their level of understanding. Adolescents and young adults with cerebral palsy need to fully understand their rights as they prepare to enter the workforce. The Americans with Disabilities Act (ADA) of 1990 (PL 101–336) states that employers with 15 or more employees shall not discriminate against a qualified individual with a disability and must provide reasonable accommodations in the workplace.117 The parents and siblings of children with cerebral palsy are also protected from discrimination under the ADA. Parents must be provided reasonable flexibility in work schedules to accommodate medical or therapy appointments. Accessible public services and transportation are mandated. Accessible transportation provides adolescents with cerebral palsy independent access to their peers and community as they make the transition to adulthood. Children with disabilities become adults with disabilities, and the pediatrician needs to guide the transition to adult care providers, some of whom may not have training, experience, or confidence in treating cerebral palsy. The aging process may exacerbate the underlying medical issues that individuals with cerebral palsy face, including gastroesophageal reflux, constipation, urinary incontinence, dysphagia, spasticity, and contractures. Adults with cerebral palsy are at greater risk for diseases of aging, such as osteoporosis, fractures caused by falling, pressure sores, and deconditioning.27,94 Musculoskeletal complications can decrease independence as individuals age.27 Nearly 70% of adults with cerebral palsy report pain of more than 3 months’ duration, typically located in the back and lower extremities.118

Scoliosis Neuromuscular scoliosis occurs in 25% of all children with cerebral palsy15 and in 60% to 75% of children with spastic quadriplegic cerebral palsy.119 The incidence increases with severity of neurologic involvement. Unlike idiopathic scoliosis, neuromuscular scoliosis is more likely to progress during adulthood and to lead to decubiti and other skin problems.119 With progression, scoliosis can contribute to pelvic obliquity, ischial decubiti, and cardiopulmonary compromise. Infrequently, progressive neuromuscular scoliosis can lead to restrictive lung disease, with

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secondary hypoventilation, increased pulmonary vascular resistance, right ventricular hypertrophy, and in extreme instances, death.120 Viewing the child while in a seated and forward flexed position, the clinician can detect asymmetries of the shoulders, spine, trunk, or pelvis in children with developing scoliosis. In ambulatory children, scoliosis is best assessed with the child in a standing and forward flexed position. Risk factors for the progression of neuromuscular scoliosis in children with cerebral palsy include curves of ⬎40 degrees before 15 years of age, nonambulatory status, and thoracolumbar curves.121 Small curves of no functional significance can be treated with observation alone, although orthopedic evaluations every 6 to 12 months are recommended. Flexible curves in young children can be positioned effectively in thoracolumbosacral orthoses (TLSOs). Although orthotic treatment usually cannot halt scoliosis progression, it can offer comfort and balance during supported sitting and may delay surgery in young children.122,123 Nonambulatory children can be provided with custom-molded seat inserts for wheelchairs rather than TLSOs. Soft spinal orthoses have not been shown to negatively affect pulmonary mechanics and gas exchange in children with scoliosis and severe cerebral palsy.30,124 Careful patient selection for surgical stabilization of neuromuscular scoliosis in children with cerebral palsy is essential. General indications for surgery include curves ⬎40 degrees in skeletally immature and 50 degrees in skeletally mature persons. Surgery is aimed at halting curve progression, improving truncal balance and sitting posture, and preventing cardiopulmonary and skin complications.119 The orthopedist selects the surgical approach on the basis of the magnitude and flexibility of the curve, typically recommending posterior spinal fusion with segmental instrumentation, with or without anterior spinal release and fusion.15,119 Spinal fusion and instrumentation for neuromuscular scolioisis in children with cerebral palsy is a major procedure, and complications can be significant. It is typically a 4- to 8-hour procedure, with prolonged general anaesthesia and 50% to 100% total blood volume losses.120 A thorough preoperative evaluation is of paramount importance. The child and family must fully understand the risks and benefits of the surgery, actively participate in goal setting, and take adequate time to opt for or against the surgery.

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Through personal interviews, 85% of the parents of children with cerebral palsy who underwent spinal fusion and instrumentation expressed satisfaction with the surgical results, commenting on the child’s increased comfort in sitting and ease of care. However, they frequently felt inadequately prepared for the experience, particularly for the intensive care environment, the degree of pain for their child, and by their child’s appearance (monitors, tubes, urinary catheters, multiple intravenous lines).125 Spinal fusion and instrumentation can be complicated during surgery by a dural tear, excessvie blood loss, pneumothorax, paraplegia, or cardiac arrest. Intraoperative blood loss can be worsened with valproate, which interferes with platelet function.119,120 Therefore, serum levels should be kept as low as therapeutically acceptable, and preoperative neurology consultation may be warranted.120 Early postoperative complications may include wound complications, pneumonia, pneumothorax, intestinal ileus, superior mesenteric artery syndrome, sepsis, and adult respiratory distress syndrome. Late complications include hardware infection or failure, curve progression, decubitus ulcers, and wound dehiscence. Comstock describes 109 postoperative complications in 79 patients, with wound infections (0% to 29%) and hardware failure (4% to 21%) being the most frequent.125 Children with quadriplegic cerebral palsy often have respiratory insufficiency associated with respiratory muscle weakness, abnormal chest wall compliance secondary to a rigid and deformed rib cage, and decreased lung compliance. Residual bronchopulmonary dysplasia, chronic recurrent aspirations leading to inflammatory reactions and fibrosis, and impaired airway clearance mechanisms secondary to upper airway abnormalities of tone and motor control can increase surgical risk.120 Moreover, spinal surgery can exacerbate gastroesophageal reflux and increase the risk of aspiration, particularly in children with decreased vital capacity.119 The child with history of recurrent pneumonia warrants careful preoperative planning. The results of chest radiographs, arterial blood gases, and pulmonary function tests should be reviewed to assess surgical risks and formulate appropriate preoperative and postoperative treatment. There are no absolute values on pulmonary function tests or blood gases that suggest surgery might be contraindicated. Aggressive preoperative pulmonary management with bronchodilators, postural drainage, and

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intravenous hydration is recommended, although evidence of outcome is not available.73 Optimizing the nutritional status of children with cerebral palsy before and promptly after spinal surgery is invaluable. The increased metabolic demands of surgical and postsurgical stressors can be significant. Children with weight for height below the 5th percentile are at increased risk for the postoperative complications of wound dehiscence, infection, and respiratory failure. Preoperative serum albumin levels of ⬎3.5 g per deciliter and total lymphocyte counts of ⬎1.5 g per liter are associated with lower rates of infection, shorter duration of endotracheal intubation, and shorter lengths of hospitalization.126 In some children, it is appropriate to consider gastrostomy tube placement, with or without fundoplication, in the months before to spinal surgery.120 The gastrointestinal tract in children with cerebral palsy is prone to dysmotility. This predisposition, when combined with a prolonged course of general anaesthesia, narcotic administration, and supine positioning, often leads to prolonged postoperative ileus. Postoperative epigastric tenderness, nausea, vomiting, and gastric distention should alert the clinician to the possibility of a superior mesenteric artery syndrome (SMA). The functional obstruction of the duodenum by the superior mesenteric artery can be demonstrated on an upper gastrointestinal series. Nasojejunal or parenteral nutrition is the mainstay of treatment for SMA.120 It is recommended that the pediatrician optimize hydration and relieve chronic constipation and fecal impaction before surgery.

Adaptive Sports Adaptive sports programs provide children with cerebral palsy opportunities to increase endurance, self-esteem, and strength in a peer setting.29 However, poor muscle strength and endurance as well as accessibility issues may limit participation. Regular physical activity reduces disease risk, promotes psychological well being, increases muscular strength and flexibility, and promotes self-image and self-efficacy during the adolescent years. Therapeutic horseback riding, or hippotherapy, improves gross motor function while also providing social and recreational opportunities.127 Any exercise prescription should be based on individual interests and abilities and should include safe, effective, and enjoyable activities.128 The intensity, frequency, and duration of physical activity necessary for improving fitness and functional health

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outcomes in persons with cerebral palsy is unknown.129

Osteoporosis and Fractures Pathologic fractures in children with cerebral palsy can result from limb rigidity, joint contractures, hip dislocations, poor balance leading to falls, violent seizures, or osteopenia. The most common site of fracture is the femur.131,132 Immobilization in casts, vitamin D deficiency, anticonvulsant therapy, and poor nutritional status increase the risk. Osteopenia, as defined by bone mineral density (BMD) z scores ⬍⫺2, was found in the femur of 97% of children with moderate to severe cerebral palsy older than 9 years of age, with fractures occurring in 26% of those older than 10 years.131 The severity of neurologic impairment, increasing difficulty feeding the child, use of anticonvulsants, and lower triceps skinfold z scores all independently contributed to lower BMD z scores in the femur.131 Although BMD is decreased in all children with cerebral palsy, it is most severely decreased in those who are nonambulatory, suggesting that immobility and lack of weight bearing is a major contributing factor.132 Among children and adults with cerebral palsy in residential care, rickets, osteopenia, and pathologic fractures are associated with vitamin D deficiency. Three months of vitamin D supplementation reduces fracture rates and improves overall clinical status.133 Similarly, supplementation of vitamin D and calcium increases BMD in children with severe cerebral palsy receiving antiepileptic medications.134 Eight months of weight-bearing activities have been shown to increase the BMD of the femur by 9.6% in children with spastic cerebral palsy.135

Life Expectancy Severity of disability is the major determinant of death of persons with cerebral palsy. Individuals with hemiplegia typically have only a 5-year reduction in the normal life span of 70 to 90 years.27 Mobility and feeding skills are found to be the most powerful prognostic indicators for survival, with independent ambulators and oral eaters having the greatest longterm survival.136 Children with cerebral palsy who require feeding tubes during the first year of life have a 5-times greater mortality rate than children with some self-feeding skills.136 The probability of se-

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verely disabled children with cerebral palsy reaching 20 years of age is only 50%.137 Respiratory infections are the leading cause of death, followed by seizure disorders and aspiration events.138 Newer advances in the treatment of individuals with cerebral palsy may influence length of survival.27

Summary and Conclusions Prematurity, an important risk factor for cerebral palsy, is not diminishing. Critical steps in development of the cortical brain occur between the 22nd and 36th weeks after conception, and research is ongoing to identify which practices optimize outcomes.5 Prematurity, however, is not the only risk factor, and current evidence supports a multifactorial basis for the disorder.6 Since presently we can neither prevent nor cure cerebral palsy, we need to optimally manage it. Parents of children with cerebral palsy look to their pediatrician for information, medical treatment, guidance, and support over years of developmental and functional transitions. However, pediatricians report a lack of training and confidence in caring for children with disabilities. More than 70% report having received no training in the prescription of durable medical equipment and more than 50% lacked training in prescribing specific therapies.139 Anticipatory guidance for the child and family is critical, from the time of diagnosis and throughout the child’s life. Parents need to know that although cerebral palsy is defined as static encephalopathy, it is not a clinically static condition. They need to know that musculoskeletal, cardiopulmonary, and digestive systems may be affected as their child grows and that children with chronic health conditions are 3 times more likely to be hospitalized when compared with the general pediatric population.140 Functionally dependent children with severe cerebral palsy and feeding tube dependency have the lowest estimated mental age, use the most medications, encounter the most respiratory problems, and miss more days of usual activities.141 The parents are the experts regarding their child and need to be empowered with knowledge and support to make the best decisions possible. The pediatrician, in cooperation with the child, family, and interdisciplinary team, can coordinate a complex care system to the maximal benefit of each child.130

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APPENDIX INTERNET RESOURCES

National Library Service for the Blind and Physically Handicapped Easter Seal/March of Dimes Children’s Hemiplegia and Stroke Association Pediatric Stroke United Cerebral Palsy Associations CP Parent Home Page The Cerebral Palsy Network Family Voices Family Village (Parent Site) Family Caregiver Alliance National Family Caregivers Association Sibling Information Network The Sibling Support Project National Parent Network on Disabilities Special Needs Advocate for Parents National Parent-to-Parent Network Exceptional Parent Resources Parents Reaching Out to Parents National Respite Locator Service Health Insurance Association of America Beneficial Designs, Inc.* Disabled Sports USA Sport Information Resource Center Special Olympics International National Ability Center (NAC) National Center on Accessibility National Office Technology Access Center Disability and Rehabilitation Research National Information Center for Children and Youth with Disabilities National Organization on Disability National Association of Developmental Disabilities Councils Disability Rights Education and Defense Fund ADA Home Page

http://www.loc.gov/nls http://www.esmodnc.org http://www.chasa.org http://www.pediatricstrokenetwork.com http://www.ucpa.org http://www.cpparent.org http://www.thecpnetwork.netfirms.com http://www.familyvoices.org http://www.familyvillage.com http://www.caregiver.com http://www.nfcacares.org http://www.uconnvm.uconn.edu http://www.thearc.org/siblingssupport http://www.npnd.org http://www.snapinfo.org http://www.netnet.net/mums http://www.eparent.com http://www.parentsreachingout.com http://www.chtop.com http://www.hiaa.org http://www.beneficialdesigns.com http://[email protected] http://www.circ.ca http://www.specialolympics.org http://www.nationalbilitiescenter.org http://www.Indiana.edu http://www.ATAccess.org http://www.resna.org http://www.nichy.org http://www.nod.org http://www.naddc.org http://www.dredf.org http://www.ada.gov

*adaptive sporting equipment

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