CURRENT Diagnosis and Treatment Pediatrics, (Current Pediatric Diagnosis & Treatment) 22nd Edition
28. Rehabilitation Medicine
Pamela E. Wilson, MD
Gerald H. Clayton, PhD
Rehabilitation medicine is the multispecialty discipline involved in diagnosis and therapy of individuals with congenital and acquired disabilities. The goals of rehabilitation medicine are to maximize functional capabilities and improve quality of life. Disabilities are described using the World Health Organization’s International Classification of Function, Health, and Disability. Three aspects are evaluated in every patient: (1) the impact of the disability on body structure and function, (2) the impact of the disability on activity and participation in society, and (3) the environmental factors with an impact on the individual’s function. These three areas are the common framework for discussion of a disabling condition and its therapy.
PEDIATRIC BRAIN INJURY
ESSENTIALS OF DIAGNOSIS
Severe head injury: Glasgow Coma Score (GCS) of < 9
Moderate head injury: GCS of 9–13
Mild head injury: GCS of 13–15
There are an estimated 475,000 emergency department visits for brain injuries per year among children from birth through 14 years of age, with 3000 deaths and 37,000 hospitalizations. Children with brain injuries may have long-term deficits and disabilities that must be identified and treated.
Brain injury is classically divided into two categories based upon the timing of the pathologic findings: primary and secondary injury.
Primary injury occurs at the time of trauma, causing focal and diffuse damage. Focal damage includes skull fracture, parenchymal bruising or contusion, extraparenchymal or intraparenchymal hemorrhage, blood clots, tearing of blood vessels, or penetrating injury. Diffuse damage includes diffuse axonal injury and edema. Consequences of primary injury, either focal or diffuse, include cellular disruption with release of excitatory amino acids, opiate peptides, and inflammatory cytokines.
Secondary injury is the loss of cellular function accompanying primary injury that results in loss of cerebrovascular regulation, altered cellular homeostasis, or cell death and functional dysregulation. A primary injury can initiate the processes of secondary programmed cell death (apoptosis), which further exacerbates the primary injury. Secondary injury may develop hours or days after the initial insult. It appears to be precipitated by elevated intracranial pressure, cerebral edema, and release of neurochemical mediators. Current treatment paradigms are focused on treating and preventing secondary injury.
Classification & Assessment of Injury Severity
Traumatic brain injury is usually categorized as open or closed. Open injuries are the result of penetration of the skull by missile or sharp object or deformation of the skull with exposure of the underlying intracranial tissues. Closedinjuries are the result of blunt trauma to the head, which causes movement (intracranial acceleration or deceleration and rotational forces) and compression of brain tissue. Brain contusions are referred to as coup (occurring at the site of injury) or contra-coup (occurring on the side of the brain opposite the injury). Rating the severity of injury and eventual outcomes is important in medical management. Included below are the two most commonly used scales relevant to clinical care of these injuries in rehabilitation medicine.
A. Glasgow Coma Scale
The Glasgow Coma Scale (GCS) is the most commonly used system to assess the depth and duration of impaired consciousness in the acute setting. The score is derived from three areas of evaluation: motor responsiveness (maximum score 6), verbal performance (maximum score 5), and eye opening to stimuli (maximum score 6). The scale has been modified for use in infants and children younger than 5 years of age, allowing for their lack of verbal responsiveness and understanding. Cumulative scores on the GCS define injury as mild (13–15), moderate (9–12), and severe (≤ 8). The concept of posttraumatic amnesia is used to gauge severity of injury and is an adjunct to the GCS, termed the GCS-E (extended). Posttraumatic amnesia is defined as the period of time after an injury during which new memory cannot be incorporated and the person appears confused or disoriented. Amnesia can be retrograde, anterograde, or both. A complicating factor in the use of either of these tools is the utilization of anesthesia, paralytics, and intubation in the acute care setting.
B. Rancho Los Amigos Levels of Cognitive Function
The Rancho Los Amigos Levels of Cognitive Function (LCFS or “Rancho”) is used to gauge the overall severity of cognitive deficit and can be used serially during recovery as a rough gauge of improvement. The scale has 10 levels of functioning ranging from “no response” to “purposeful, appropriate.”
Common Sequelae of Brain Injury
Depending on the severity of brain injury, there may be deficits in cognition and behavior, as well as physical impairments. Injuries can also produce changes in sensory and motor function, emotional stability, social behavior, speed of mental processing, memory, speech, and language. The consequences of mild brain injuries may be difficult to discern. Small intraparenchymal injuries, easily identified by computed tomographic (CT) or magnetic resonance imaging (MRI) scans, may not cause obvious signs or symptoms. The following are common problems associated with brain injury.
Seizures occurring in the first 24 hours after injury are referred to as immediate seizures. Those occurring during the first week are early seizures, and those starting more than 1 week after injury are referred to as late seizures. Seizure prophylaxis with medications is recommended in the first week after brain injury in children at high risk for seizures and in very young children, who are at higher risk for early seizures than are older children and adults. Seizure prophylaxis is also recommended for 1 week after any penetrating brain trauma. Seizure prophylaxis is probably not effective for prevention of late-onset seizures. Late-developing seizures may require long-term treatment.
B. Neuromotor Deficits/Movement Disorders
Neuromotor deficits after brain injury include movement disorders, spasticity, paralysis, and weakness. The type of disorder will be influenced by the areas damaged from the trauma. The most common movement disorders are tremors and dystonias. These deficits can result in impaired ambulation, coordination, impaired ability to use upper extremities, and speech problems. Physical therapy is the primary means of treating these problems.
C. Communication Disorders
Language and communication disorders are fairly common. Aphasia, which is difficulty in understanding and producing written and spoken language, is categorized as fluent, nonfluent, or global. Individuals with fluent aphasia or Wernicke type disorder can produce speech, but have little content associated. The nonfluent aphasias or Broca’s type have a paucity of speech and may have word finding difficulties. Global aphasias have extensive injuries and the most severe language disorders.
D. Paroxysmal Sympathetic Hyperactivity
Severe brain injuries may be associated with excessive sympathetic outflow and results in a constellation of symptoms known as paroxysmal sympathetic hyperactivity (PSH). Symptoms of PSH are tachycardia, tachypnea, sweating, hyperthermia, hypertension, agitation, and posturing. Common medications used to treat PSH include dopamine agonists (eg, bromocriptine), β-blockers (eg, propranolol), and α-agonists (eg, clonidine).
E. Cognitive and Behavioral Deficits
After brain injury, cognitive and behavioral deficits are a frequent occurrence. Cognitive disorders depend on the location and severity of the injury. Damage to the frontal lobes can cause executive function problems along with initiation delays. Neuropsychiatric sequelae are common, and depression, anxiety disorders, and posttraumatic stress disorders (PTSD) are present in one-third of those injured. Testing by a neuropsychologist may help to identify problem areas and develop interventional programs including school modifications and behavioral strategies.
F. Hypothalamic-Pituitary-Adrenal Axis Dysfunction
Dysfunction of the hypothalamic-pituitary-adrenal axis is common after head injury. The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and diabetes insipidus (DI) from a posterior pituitary injury can result in significant electrolyte and osmolality imbalance. Amenorrhea that typically resolves spontaneously is common in females. Injury near the onset of puberty can complicate normal development, and endocrine status should be monitored closely.
G. Cranial Nerve Injuries
The sensory and motor components of the cranial nerves are often damaged, resulting in a wide variety of deficits not centrally mediated. The most commonly injured nerves are I, IV, VII, and VIII. Hyposmia or anosmia (cranial nerve I) can occur if the shearing forces at the cribriform plate disrupt the afferent olfactory nerves. Injury to cranial nerves IV (trochlear) is common as it has the longest intracranial length. Superior oblique injuries typically cause a head tilt and vertical diplopia. Facial nerve injuries (cranial nerve VII) are common especially with temporal bone fractures. This impacts the ability to use the facial muscles, causes dryness in the eye and salivary glands along with decreasing taste in the anterior part of the tongue. The cochlear nerve (VIII) is also frequently damaged in temporal bone fractures and can result in vertigo and dizziness.
Much of what we know of traumatic brain injury is based on adult experience. The confounding effects of age and the etiologies unique to the pediatric population (eg, child abuse) make care of the pediatric head-injured patient very complex.
The assumption that younger children will fare better than older children or adults after a brain injury is a myth. The fact that in a child a significant amount of development and synaptic reorganization has yet to occur does not guarantee an improved chance for functional recovery. Indeed, disruption of developmental processes, especially in very young infants or neonates, may be catastrophic. These processes often cannot be resumed once disrupted.
The mechanism of injury plays an important role in determining the severity of brain injury in very young children. Mechanisms associated with nonaccidental injury such as shaking often result in global diffuse injury. The weak neck musculature, large head-body mass ratio, immature blood-brain barrier, and high intracranial fluid–brain mass ratio all contribute to widespread damage.
During puberty, major hormonal changes have an impact on the outcome of brain injury. Behavioral problems may be pronounced in brain-injured adolescents. Precocious puberty and precocious development of sexual activity may occur in preadolescents and should be carefully monitored.
Careful consideration should be given to the developmental progress of the brain-injured child and adolescent. Delays can be anticipated after moderate and severe brain injuries related to abnormalities of cognition and behavior. It is critical to identify developmental disabilities as early as possible so that appropriate therapy can be started in order to maximize the child’s residual capabilities. Educational programs should include an individualized educational program (IEP) to support the child with significant remediation and assistance needs during their school years. Programs should also include a 504 plan (named for Section 504 of the Rehabilitation Act and the Americans with Disabilities Act). The 504 plan identifies the accommodations necessary in regular school settings for students with lesser disabilities so that they may be educated in a setting with their peers.
The primary goal of rehabilitation after childhood brain injury is to maximize functional independence. Rehabilitative care can be divided into three phases: acute, subacute, and long term. The acute and subacute phases typically occur in the inpatient setting while the long-term phase is an outpatient endeavor.
A. Acute Care
Therapy in the acute phase consists mainly of medical, surgical, and pharmacologic measures to decrease brain edema, treat increased intracranial pressure, and normalize laboratory values. Nutrition is essential in the healing process and either parenteral nutrition or supplemental enteral feedings are employed. Current research suggests that transitioning to enteral nutrition (eg, nasogastric tube feeding) as soon as possible after brain injury is associated with improved outcomes. Placement of a gastrostomy tube for supplemental enteral feeding is often performed in patients with severe brain injuries when recovery will be protracted and swallowing function is inadequate for safe oral feeding.
B. Subacute Care
Therapy in the subacute phase is characterized by early, intensive participation in rehabilitative therapies promoting functional recovery. Treatment should be planned after consultation with physical therapy, occupational therapy, speech-language specialists, and neuropsychologists. Nursing staff members are a primary interface with the patient and often serve as educators for family-directed care. Most children and adolescents with brain injuries can be discharged home to continue with treatment on an outpatient basis.
C. Long-Term Care
Long-term follow-up starts immediately after discharge. Medical issues must be thoroughly and regularly reviewed to ensure that changing needs are met. Annual multidisciplinary evaluation is important, especially as the child approaches school age. Neuropsychological testing may be required to define cognitive and behavioral deficits and plan strategies to deal with them in the educational environment. Therapies should be flexible, providing strategies to maximize independence and facilitate the child’s involvement in changing environments.
Medication is often required for cognitive and behavioral issues. Attention deficit and fatigue associated with brain injury may be amenable to treatment with stimulants such as methylphenidate and modafinil. Dopaminergic agents such as amantadine, levodopa, and bromocriptine can be useful in improving cognition, processing speed, and agitation. Antidepressants such as selective serotonin reuptake inhibitors can be helpful in treating depression and mood lability. Anticonvulsants can be useful as mood stabilizers and in treating agitation and aggression. Tegretol and valproic acid are typical agents for this purpose.
Attention and arousal can also be successfully addressed by utilizing behavioral techniques to reinforce desired behaviors as well as identifying environmental situations that optimize those behaviors. Gains made in the behavioral realm often have a positive impact on therapies designed to address physical issues.
Prognosis & Outcomes
Directly after brain injury, poor pupillary reactivity, low blood pH, absence of deep tendon reflexes, and low GCS all correlate with poor outcome. An increased number of intracranial lesions, and the merging of multiple smaller lesions into one, is associated with increased injury severity and poorer outcome. Increased depth and duration of coma are also associated with poor functional recovery. Children younger than 1 year of age tend to have worse outcomes.
Functional outcome assessment is important for judging the efficacy of rehabilitation therapy. Global multidomain measures (eg, FIM/WeeFIM, FRESNO) are used to provide a functional “snapshot in time” of select functions—motor function and mobility, self-care, cognition, socialization, and communication. Sensitive and specific domain-specific outcome tools are important to follow for functional recovery that may occur in small increments. Simpler, single-domain, functional assessment tools such as the Glasgow Outcome Scale (GOS) and its pediatric cousin the Kings Outcome Scale for Childhood Head Injury (KOSCHI) may also be of use.
Outcome associated with mild brain injury is often quite favorable. Most patients recover normal function within a short time. A small percentage develop persistent problems such as chronic headache, poor focusing ability, altered memory, and vestibular abnormalities, and full recovery may last for many weeks or months. Differentiating between musculoskeletal and central nervous system (CNS) etiologies of symptoms associated with these types of injuries (eg, headache) is important and can influence prognosis and care planning.
In children, recovery may not be fully achieved for many months or years after the initial injury. The impact of the injury on developmental processes and its future consequences are difficult to predict. Long-term follow-up is required, particularly as the child approaches school age.
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SPINAL CORD INJURY
ESSENTIALS OF DIAGNOSIS
Spinal cord injury (SCI) is an alteration in normal motor, sensory, or autonomic function secondary to spinal insult.
Characterized as either complete (total loss of function) or incomplete (some preservation of function below the level of lesion).
Epidemiologic studies of spinal cord injuries (SCI) suggest that there will be about 10,000 new injuries per year and that 20% will be in those younger than age 20 years. Motor vehicle accidents are the leading cause of SCI in all ages. Falls are common causes in young children. The phenomenon of SCI without obvious radiologic abnormality (SCIWORA) can be present in 20%–40% of young children. Children from birth to 2 years tend to have high-level injuries to the cervical spine because of the anatomical features of the spine in this age group. The facets tend to be shallower and oriented horizontally, and the boney spine is more flexible than the spinal cord. In addition, the head is disproportionately large and the neck muscles are weak.
A. Classification and Assessment of Injury Severity
SCI is classified using the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI), which was formerly known as the American Spinal Injury Association (ASIA) classification system.
This classification evaluates motor and sensory function, defines the neurologic level of the injury, and assesses the completeness (level of motor or sensory sparing) of the deficit. The 72-hour examination is used in predicting recovery. A complete lesion identified on examination at 72 hours predicts a poor recovery potential. The ISNCSCI classification is as follows:
1. Class A is a complete SCI, with no motor or sensory function in the lowest sacral segments.
2. Class B is an incomplete injury, with preserved sensory function but no motor function in the sacral segments.
3. Class C is an incomplete lesion in which the strength of more than 50% of key muscles below the injury level is graded less than 3/5 on manual muscle testing.
4. Class D is an incomplete lesion in which the strength of more than 50% of key muscles below the injury level is graded greater than 3/5 on manual muscle testing.
5. Class E is an injury in which full motor and sensory function is preserved.
B. Clinical Patterns of Spinal Cord Injury
1. Brown-Séquard injury—The cord is hemisected causing motor paralysis, loss of proprioception and vibration on the ipsilateral side, and loss of pain and temperature on the contralateral side.
2. Central cord syndrome—Injury is to the central part of the cord and results in greater weakness in the arms than the legs.
3. Anterior cord syndrome—Disruption of the anterior spinal artery causes motor deficits and loss of pain and temperature sensation. Proprioception and fine touch is spared.
4. Conus medullaris syndrome—Injury or tumor of the conus, the lower conical shaped end of the spinal cord, can cause minimal motor impairment but significant sensory, bowel, and bladder abnormalities.
5. Cauda equina syndrome—Injury to the nerve roots produces flaccid bilateral weakness in the legs, sensory abnormalities in the perineum, and lower motor neuron bowel and bladder dysfunction.
The diagnosis and anatomic description of SCI is made mainly through imaging techniques. Initial studies should include radiographs of the entire spine (including cervical spine) and special studies for boney structures. MRI imaging is required to evaluate soft tissues. CT scans, including three-dimensional reconstructions, may be used to further define the injured elements.
A. Initial Management
The two primary precepts of SCI treatment are early identification and immediate stabilization of the spine. The approach used to stabilize the spine is determined by the type of injury, location of injury, and underlying condition of the spinal cord. Stabilizing the spine may prevent further damage to the spinal cord. External traction devices such as halo traction and orthotics are often used. Some injuries require internal stabilization. The benefit of methylprednisolone administration in acute SCI has recently come into question. Based on the ongoing controversy regarding efficacy and outcomes, steroids remain an option, but their administration is not considered standard of care. When used, the initial loading dose is 30 mg/kg over 15 minutes, followed by 5.4 mg/kg for the next 23 hours if started within 3 hours of injury. If started within 3–8 hours of injury, corticosteroids should be continued for 48 hours.
B. Functional Expectations after Spinal Cord Injury
The lesions associated with SCI have a predictable impact on motor and sensory function. It is helpful to understand these concepts when discussing functional expectations with patients and parents (Table 28–1).
Table 28–1. Functional expectations related to spinal cord injury.
C. Special Clinical Problems Associated With Spinal Cord Injury
1. Autonomic hyperreflexia or dysreflexia—This condition occurs in spinal injuries above the T6 level. Noxious stimuli in the injured patient cause sympathetic vasoconstriction below the level of injury. Vasoconstriction produces hypertension and then a compensatory, vagal-mediated bradycardia. Symptoms include hypertension, bradycardia, headaches, and diaphoresis. This response may be severe enough to be life threatening. Treatment requires identification and relief of the underlying noxious stimulus. Bowel, bladder, and skin problems are the most common noxious stimuli causing this syndrome. The patient should be placed in an upright position and antihypertensive medication used if conservative measures fail. Nifedipine (oral or sublingual) and nitrates have been used in the treatment of this condition.
2. Hypercalcemia—Hypercalcemia often occurs in male adolescents within the first 2 months of becoming paraplegic or tetraplegic. The serum calcium level rises significantly in response to immobilization. Patients complain of abdominal pain and malaise. Behavioral problems may occur. Initial treatment is focused on hydration and forced diuresis using fluids and furosemide to increase urinary excretion of calcium. In severe cases, especially in older children, calcitonin and etidronate may be required.
3. Thermoregulation problems—These problems are most common and most severe in higher level injuries and usually result in a poikilothermic state where body temperature changes with that of the environment. The ability to vasoconstrict and vasodilate below the injury level is impaired. The person with an SCI above T6 is particularly susceptible to environmental temperature and is at risk for hypothermia and hyperthermia.
4. Deep vein thrombosis—Thrombosis is a common complication of SCI, especially in postpubescent children. Deep vein thrombosis should be suspected in children with any unilateral extremity swelling, palpable cords in the calf muscles, fevers, erythema, or leg pain. Diagnosis is confirmed by Doppler ultrasound, and full evaluation may require spiral CT scan or ventilation-perfusion scan if pulmonary embolus is suspected. Preventative measures include elastic stockings and compression devices. Anticoagulation prophylaxis may be required using medications such as low-molecular-weight heparins (eg, enoxaparin, 0.5 mg/kg subcutaneously, every 12 hours).
5. Heterotopic ossification—This complication occurs in both spinal cord and traumatic brain injuries. Ectopic calcium deposits usually appear around joints in the first 6 months after injury. They may cause swelling, decreased range of motion, pain with motion, palpable firm masses, fever, elevated sedimentation rate, and abnormal triple phase bone scan. Nonsteroidal anti-inflammatory drugs or bisphosphonates such as Etidronate should be started at the time of diagnosis. Surgical removal of ectopic deposits is controversial and usually performed only in cases of extreme loss of motion, pressure sores, or severe pain.
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BRACHIAL PLEXUS LESIONS
ESSENTIALS OF DIAGNOSIS
Upper trunk (C5 and C6) is the most common area injured and results in the classic Erb’s palsy.
Injury to the lower trunk (C7–T1) produces a Klumpke palsy.
Pan plexus lesion involves all roots.
Brachial plexus lesions associated with delivery are related to traction applied to the nerves and is often associated with shoulder dystocia. The nerve injury can range from simple neuropraxia (stretch) to complete avulsion. Acquired brachial plexus injuries from sports, surgery, and accidents also have a mechanism which stretches or injures the plexus.
Identification of factors associated with shoulder dystocia, such as macrosomia, or proper positioning during surgical procedures to decrease traction on the brachial plexus may reduce the incidence of these lesions.
Erb’s palsy has been described as the “waiter’s tip posture” and is characterized by shoulder weakness with internal rotation and adduction of the upper arm. The elbow is extended and the wrist flexed. There is good preservation of hand function. Klumpke palsy is characterized by good shoulder function but decreased or absent hand function. Brachial plexus injuries may also cause a Horner’s syndrome (unilateral miosis, ptosis, and facial anhydrosis) due to disruption of cervical sympathetic nerves. Associated boney and nerve injuries are common in brachial plexus injury. The physical examination should include inspection of the humerus and clavicle for fractures. There may be injuries of the phrenic and facial nerves. The diagnosis of a brachial plexus lesion should be based on the history and clinical examination. Diagnostic testing helps confirm, localize, and classify the lesion. Electromyography is helpful 3–4 weeks after the injury. This test not only is used diagnostically but also can track recovery. MRI, myelography, and CT scan can help to locate the lesion and determine its extent.
The development of complications reflect the degree of nerve recovery. Severe injuries are at risk for shoulder contractures, muscle atrophy, osseous deformities, functional deficits, pain, and maladapted postures.
Treatment & Prognosis
The treatment for brachial plexus lesions will depend on the severity of the lesion. Many will heal on their own and no interventions are needed. For persistent injuries, physical/occupational therapy is the major treatment and includes stretching, bracing, strengthening, electrical stimulation, and functional training. Primary surgery to the nerves of the plexus is indicated for children who have no spontaneous recovery of biceps function by 6–9 months. Secondary procedures to maximize function include muscle transfers and orthopedic interventions.
Many factors are used to predict recovery. The anatomic location of the lesion impacts recovery, as upper trunk lesions do better than lower trunk lesions. If a Horner’s syndrome is present, these injuries always have a poor recovery. Children in whom antigravity function returns within 2 months of injury will usually have a good recovery of function. If antigravity function is delayed until 6 months, recovery will probably be limited. If antigravity function is absent at 6–9 months, there will be no recovery of function and surgery should be considered.
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COMMON REHABILITATION PROBLEMS
1. Neurogenic Bladder
The muscles of the bladder include the detrusor and urethral sphincters. During the first year of life the bladder is a reflex-driven system that empties spontaneously. After the first year control begins to develop, and most children achieve continence by age 5 years. Children with damage to the central or peripheral nervous system may develop a neurogenic bladder. Neurogenic bladder is usually classified as noted below:
1. Uninhibited neurogenic bladder occurs after upper motor neuron injuries at the level of the brain or spinal cord that result in failure to inhibit detrusor contractions. This results in a hyperreflexive voiding pattern.
2. Reflex neurogenic bladder results from damage to the sensory and motor nerves above the S3 and S4 level. The bladder empties reflexively but coordination may not be present and dyssynergia (contraction of the bladder musculature against a closed sphincter) can occur. Increased intravesicular pressure and vesicourethral reflux may be consequences of dyssynergia.
3. Autonomous neurogenic bladder is a flaccid bladder and is associated with lower motor neuron damage. Bladder volumes are usually increased and overflow incontinence can occur.
4. Motor paralytic neurogenic bladder results from injury to the motor nerves of the S2–S4 roots. Sensation is intact but there is motor dysfunction. The child has the sensation to void but has difficulty with the voluntary contractions.
5. Sensory paralytic neurogenic bladder results when sensory roots are disrupted. Affected patients do not have sensation of the full bladder but are able to initiate voiding.
The diagnosis of neurogenic bladder requires a complete history and physical examination. The type of neurologic damage should be identified as this will help to predict anticipated voiding issues. The upper tracts should be assessed by several techniques, including ultrasound, intravenous pyelogram, and renogram (isotope) studies. Lower tract testing includes urinalysis, postvoid residuals, urodynamics, cystography, and cystoscopy.
Treatment is geared to the type of bladder dysfunction. The simplest methods are those employing behavioral strategies. Timed voiding can be effective for children with uninhibited bladders. In this technique, children are reminded verbally or use a cueing device (watch with a timer) to void every 2–3 hours before bladder capacity is reached. The Credé and Valsalva maneuvers are used in patients with an autonomous bladder to assist in draining a flaccid bladder. There is a risk of increasing intravesicular pressure during these maneuvers, which can provoke vesicoureteral reflux. These maneuvers should never be used in a patient with a reflex neurogenic bladder.
Medications are often employed to treat neurogenic bladder. Anticholinergics are commonly used to reduce detrusor contractions, decrease the sense of urgency, and increase bladder capacity. Medications include oxybutynin, tolterodine, and hyoscyamine. The side effects of these medications include sleepiness, nausea, and constipation. External methods are also used to improve continence and function. Absorbent pads and diapers, external catheters, internal catheters, and intermittent catheterization are some typical methods. Surgical procedures to protect the upper tracts from urinary reflux are often used. A young child with a high-pressure bladder is at particular risk for reflux and may need medication, intermittent catheterization, or vesicostomy to prevent hydrostatic renal damage and infection. An older child may require reconstructive bladder surgery (bladder augmentation) to increase the capacity of the bladder or an intestinal conduit from bladder to skin surface (Mitrofanoff procedure) to relieve bladder distention. If an incompetent urethral sphincter causes urinary leakage, injections, slings, or implants may be used to increase the urethral barrier. Recently, electrical stimulation of sacral roots has been used to initiate voiding. Biofeedback training is also used to improve voiding.
2. Neurogenic Bowel
Control of bowel function depends on an intact autonomic (sympathetic and parasympathetic) and somatic nervous system. Interruption of any of these pathways can result in retention and/or incontinence. Goals of treatment for patients with neurogenic bowel are to establish a predictable and reliable bowel habit, and prevent incontinence and complications. There are two types of neurogenic bowel dysfunction: upper motor and lower motor dysfunction. The upper neuron bowel results from damage above the conus. Affected patients usually have reflex bowel contractions of high amplitude, absence of sensation, and no voluntary sphincter control. Patients with the lower motor neuron bowel have no voluntary sphincter control and no reflex contraction of the external anal sphincter (anocutaneous reflex). It has been described as a flaccid bowel. In general, establishing a bowel program is easier in patients with upper motor neuron lesions.
Diet is important in either type of neurogenic bowel. Fiber and fluids are critical elements. Stool consistency should be on the soft side, although some patients try to keep themselves constipated to prevent accidents. A predictable and scheduled bowel program is essential. Bowel movements should be scheduled to occur with meals, as the gastrocolic reflex can trigger defecation.
Laxative and stool softening medications are usually included in a comprehensive bowel program. Stool softeners such as docusate retain stool water. Mineral oil is an acceptable stool softener in patients not at risk for pulmonary aspiration. Bulking agents such as Metamucil increase fiber and water content of stool and reduce transit time. Stimulants such as senna fruit extract or bisacodyl increase peristalsis. Osmotic agents such as polyethylene glycol keep stools soft by retaining stool water. Suppositories and enemas are often used when other methods have not been successful. Upper motor neuron bowel management programs may include digital rectal stimulation. When conservative methods are ineffective, options may include surgical implantation of sacral nerve stimulators or techniques to facilitate antegrade flushing of the colon. For example, the ACE (antegrade continence enema) or Malone procedure approximates the appendix to the surface of the abdomen providing a conduit for flushing. Also, a capped cecostomy tube can be placed into the cecum through which fluids can be administered in an antegrade fashion to remove fecal matter from the colon.
Spasticity is a velocity-dependent increase in muscle tone and a loss of isolated muscle function. Whereas tone is the resistance felt in a muscle as it is moved in space, spasticity occurs when there is damage to the CNS from trauma or injury. It is included in the upper motor neuron syndrome (hyperactive and exaggerated reflexes, increased tone, clonus, positive Babinski sign). Spasticity is evaluated using the Ashworth Scale, with 0 indicating no increase in muscle tone and 4 indicating complete rigidity of the extremity.
Treatment is goal directed and influenced by the functional status of the client. Spasticity has both positive and negative effects on quality of life. The positive aspects include the ability to use spasticity for functional tasks along with maintaining muscle strength. Negatively spasticity can interfere with positioning and hygiene, can affect function, and can cause pain.
Options for therapy range from conservative to aggressive. A pyramidal approach starts from a base of prevention of nociceptive input and aggressive physical therapy. Children should be positioned properly and have appropriate equipment to support this strategy. Physical therapy is designed to reduce the long-term effects of spasticity by stretching and range-of-motion exercises. Heat and cold are useful in improving tone, but their effects are not long lived. Casting of both upper and lower extremities can decrease tone and increase range of motion. Constraint therapy can be used to try to improve upper extremity function.
The next step on the pyramid is the use of medications, mainly baclofen, diazepam, dantrolene, and tizanidine. Baclofen (a direct GABAB agonist) is a first-line medication, which produces effects at the spinal cord level. Side effects are primarily sleepiness and weakness. Seizure threshold may be reduced by baclofen. Baclofen can be delivered directly to the CNS through an intrathecal pump. It has been used successfully in children with brain injury, cerebral palsy, and SCI. Diazepam is an allosteric modulator of postsynaptic GABAA receptors in both the brain and the spinal cord. It can cause drowsiness and dependence. Dantrolene decreases the release of calcium in muscle. Side effects include weakness and, rarely, hepatotoxicity. Tizanidine is a newer agent and works at the α2-adrenergic receptors presynaptically. It can cause dry mouth and sedation, and liver function tests can be elevated.
Relief of focal spasticity can be achieved by using chemodenervation techniques. Botulinum toxin A and B can be injected in selected muscles to improve range of motion, thus improving function and hygiene as well as reducing pain and deformity. More recently, botulinum toxins have been used to treat drooling, hyperhydrosis, and chronic pain. These toxins block the release of acetylcholine at the neuromuscular junction. The effects are temporary, lasting only 3–6 months, and repeat injections are often needed. Phenol injections are another option for treatment of local spasticity and are technically more challenging. Phenol denatures proteins in both myelinated and unmyelinated fibers and produces neurolysis or myolysis, depending on the site of injection. The effects may last longer than botulinum toxins. Injections carry a risk of sensory dysesthesia if mixed nerves are injected.
Surgical options include orthopedic procedures geared toward improving function and ambulation and alleviating deformities produced over time by spasticity. Contractures are common in the Achilles tendon, hamstrings, and adductors. Upper extremity contractures occur in the elbow, wrist, and finger flexors. Scoliosis is fairly common and bracing or surgery may be needed. Gait analysis may be helpful in evaluating the child with functional spasticity as a guide for the use of orthotics, therapy, and surgery. Neurosurgical techniques such as selective dorsal rhizotomy, sectioning afferent sensory nerve fibers, are used in a very select group of children to permanently alter spasticity patterns and improve ambulation.
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QUALITY ASSURANCE/IMPROVEMENT INITIATIVES IN REHABILITATION MEDICINE
Working with the Accreditation Council for Graduate Medical Education (ACGME), the American Academy of Physical Medicine and Rehabilitation (AAPM&R) fosters acquisition of knowledge regarding the use of quality assurance/improvement techniques in its training programs. This is now one of six competencies required for board certification. The AAPM&R feels that these skills endow the practitioner with the capacity to maintain and improve the quality of care provided to the public.