Children with special health care needs (SHCNs) represent a growing population of patients with unique needs and are important from a health care services and policy perspective. Physiatry as a specialty is uniquely suited to take a primary role in the management of SHCN children and can play a major part in limiting the degree of impairment and disability that these children are at risk of attaining. Of importance to the physiatrist is an understanding of the role and impact that physical and cognitive development has on common physiatric problems and how these problems may differ in the pediatric population versus the adult population. This chapter seeks to define many of the common issues addressed by the pediatric physiatrist and points out important aspects of these issues that are unique to the pediatric population.

As defined by the federal Maternal and Child Health Bureau, SHCN children are those who are at increased risk for a chronic physical, developmental, behavioral, and emotional condition and who also require health care–related services of type or amount beyond that required by children in general.¹ A number of studies over the past 10 to 15 years have attempted to define the prevalence of childhood disabilities. An estimated 12.8% of children in the United States met criteria for SHCNs in 2001, with a substantial percentage of them (17%) having unmet health care needs.² Boyle et al demonstrated an increase in the prevalence of developmental disabilities from 12.8% to 15% from 1997 to 2008.³ Of further concern is the effect that childhood disability has on the individual’s health as he or she ages and the implications this holds as the child transitions to adult-based care. A recent study assessing adult-based outcomes in individuals with cerebral palsy (CP) and spina bifida (SB) showed an increased incidence of chronic pain (59%) and progressive deterioration of functional ambulatory skills (44%) in participants with CP and poor aerobic fitness (80%) in individuals with both CP and SB.⁴ Additional studies are clearly needed to further define the physical and functional changes seen by SHCN children as they age.

The physical complications encountered by children with physical disabilities are similar to those seen in adults with physical disabilities, and general management strategies for those complications tend to be similar. However, key differences between children and adults exist and may necessitate modifying those strategies, or applying additional considerations, in order to effectively treat the patient. These differences are mostly secondary to childhood developmental considerations, including body growth, cognitive development, neuromotor development, and cardiovascular adaptation.

Tone and Spasticity

There are many disorders of tone and movement that lead to complications in pediatric rehabilitation; a few common types will be discussed in this section. Spasticity is an involuntary, velocity-dependent increase in muscle tone and is typically the result of loss of pyramidal upper motor neuron suppression of the lower motor neurons and spinal reflexes (Fig. 64–1).

Severity ranges from mild to severe, and muscles involved range from isolated muscles to diffuse involvement. Although spasticity can affect any muscle, there are common synergistic patterns of spasticity in the extremities. In the upper extremity, a common pattern is adduction, internal rotation, and flexion at the shoulder, elbow flexion, forearm pronation, wrist flexion with ulnar deviation, finger flexion, and thumb in palm. In the lower extremity, the most common pattern is hip flexion and adduction, knee flexion, and equinus or equinovarus positioning of the foot, possibly with toe flexion and/or great toe extension.

Ataxia and dystonia each have a very broad group of etiologies, have varied presentations, and can affect focal body regions or global function. Ataxia is difficulty with coordinated voluntary muscle movements. Dystonia is a syndrome of involuntary intermittent or sustained muscles contraction involving co-contraction of both agonist and antagonist muscles. There are many other types of tone and movement disorders, some include hypotonia, mixed tone, chorea, athetosis, ballism, myoclonus, apraxia, and so on.

Disorders of tone and movement typically impair the ability of the body or body part to move normally and affect how the rest of the body moves and compensates to accomplish tasks. Most often these disorders will challenge the child’s gross, fine, and oral motor skills. For skills already attained, these tasks continue to require increased time and energy expenditure and often cause pain and fatigue. As the child ages, new functional gains often require increasing physical and cognitive abilities. As these challenges become more significant, milestones may be delayed or may first be accomplished in an atypical manner. Further milestones may be too challenging to be performed without modification, bracing/equipment, and/or therapy training or assistance. For instance, a child with significant lower extremity tone may be able to roll over at a typical developmental age, but crawling may be delayed or performed as army crawling, which is still an effective means of exploring the household environment. Although army crawling might be accomplished independently, ambulation might require bracing and equipment, as well as physical therapy to direct gait training. In some cases (more often with spasticity), increased tone can bolster some aspects of function, providing active support through the limbs or trunk. For example, increased trunk tone may improve sitting balance, increased lower extremity tone may make standing transfers easier or perhaps improve ambulation, and increased upper extremity tone could aid in holding objects by increasing grip.

Over time, abnormal movement patterns due to abnormal tone can lead to functional limitations in motor development, bone and joint deformities, increased risk for early development of osteoarthritis, nerve entrapment, and so on. Increased tone in muscles can cause their joints of action to lose range of motion (ROM) and, if sustained, can lead to the development of joint deformity, contractures, and worsening functional impairments. Looking at crouch gait as an example, over time the complications of this gait pattern can become more pronounced with an increase in energy demand of ambulation, decrease in functional distances that can be walked, and increase in failures of the gait pattern with subsequent falls. In particular, bone and joint deformity (most importantly in the lower extremities) that results from tone and abnormal functional mechanics can lead to lever arm dysfunction in both the hips and feet, further decreasing the strength and function of already impaired muscles.

Hydrocephalus

Hydrocephalus is an impairment in the production, absorption, or flow of cerebrospinal fluid (CSF) within the brain. This typically leads to increased size of the ventricular system and increased diffuse intracranial pressure (typically if the lateral ventricles are enlarged) and/or focal pressure (typically if the third and fourth ventricles are enlarged) on adjacent nervous system tissue. The condition often acutely results in symptoms including headache, decreased level of awareness and cognition, emesis, gait imbalance, possibly increased lower extremity spasticity, and blurred or double vision. Infants with hydrocephalus often present with the “setting-sun” phenomenon with the eyes driven downward (Fig. 64–2).

In pediatric rehabilitation, hydrocephalus is typically secondary to another condition or injury, and the primary condition is important in how hydrocephalus affects function through growth and development. With traumatic brain injury or prematurity, this most often is secondary to intracranial hemorrhage. The development of posttraumatic hydrocephalus most likely prognosticates a poorer outcome in all areas of recovery, especially with cognitive and emotional/behavioral recovery (memory, attention, and irritability). In patients with myelomeningocele, those with hydrocephalus demonstrated impaired executive function, memory, and processing speed in comparison with relatively normal neuropsychological function in those without hydrocephalus.⁵

Dysphagia

Dysphagia is a common issue in children with SHCNs that can have a significant effect on overall health, growth, and development. The primary challenge with dysphagia is safely providing the child with adequate nutrition, hydration, and necessary medications while minimizing the risk of aspiration-associated infections and other complications. Adequate nutrition is vital for all children, especially for those with disabilities, who may have higher caloric needs or special nutrition requirements and may be more medically susceptible to illness. Additionally, for most families or caregivers, it is important to provide appropriate nutrition in a manageable amount of time. Critical in evaluating swallow function is prescribing a safe and time-efficient means for meeting enteral needs. When compensations for dysphagia are inadequate (i.e., body positioning, compensatory mechanisms such as the chin tuck, texture of solids and liquids, size of bites or size of straws for liquids, pacing of meals, etc.), the primary short-term risk is pulmonary infections such as aspiration pneumonia. Over a longer amount of time, inadequate compensation for dysphagia can lead to resistance to oral intake or inability to take adequate quantities, which can have a negative impact on physical growth, developmental gains across all areas of development, and overall state of health, possible reducing quality of life and life expectancy.

Seizures

The effect of seizures on the growing and developing child is significantly varied depending on many factors. Current age of the child is a critical factor in determining potential detrimental effects on the child.⁶ Other important factors include age at onset of seizures; the location and type of seizures; whether they are focal or generalize; the frequency, progression, or change in seizures over time; and response to and side effects of medications and treatments (Fig. 64–3).

Seizures affect the ability of the young brain to function and can negatively affect the process of typical physiologic brain development. Prolonged seizures or status epilepticus may directly injure the brain. Febrile seizures, in contrast, have not been shown to cause long-term disability. Common complications resulting from seizures include intellectual and learning disability, behavioral and social concerns, and impairments in movement and motor control, tone, posture, and balance. Within this broad spectrum of possible deficits, there is a wide range of potential individual impairment.

Focal or partial seizures without underlying structural abnormality (e.g., cortical dysplasia or tumor) may have more focused complications relating to the function of the involved part of the brain and the other areas with which it works closely. In comparison, seizures that are frequent and generalized or specific seizure types such as electrical status epilepticus in sleep (ESES) or infantile spasms typically cause delay in all areas of development and sometimes cause regression. Response to treatment is also an important factor in the breadth and severity of impacts on development. Seizure medications, while serving their important purpose, also can have important impacts on development. With this in mind, epilepsy that responds to monotherapy is likely to have fewer developmental complications than epilepsy that requires treatment with multiple medications or has an incomplete response.

Musculoskeletal

Soft tissue contractures, osteoporosis, and heterotopic ossification can all be seen in children with physical impairments. Contractures are common in children with spasticity and neuromuscular disorders and often lead to significant functional impairments. The treatment options for contractures are similar to those used in the adult population. These management options include passive and active ROM, positioning, splinting, bracing, serial casting, pharmacologic treatment of any underlying spasticity, and surgical interventions.

Surgical techniques commonly used to address contractures include tenotomies, tendon lengthenings, and musculotendinous recessions. Debate exists as to whether recessions lead to better functional outcomes than do lengthenings or tenotomies.^7,8 The risk of contracture recurrence postoperatively is also high in children due to linear body growth. Most studies suggest that the risk of recurrence is reduced if surgeries are performed after the age of 6 years.^9,10 Botulinum toxin injections in conjunction with aggressive ROM and positioning programming are commonly used to either reduce contractures or maintain ROM until optimal surgical timing is achieved.