EARLY TREATMENT
Spinal Stabilization
Once it has been determined that the child has an SCI, the spine must be stabilized. The halo external skeletal fixation device was first described in 1968 for use in adults with cervical fractures by Nickel and colleagues (17).
It has subsequently been adapted for use in children, with modifications required by the unique characteristics of the child’s skull, which is thinner. Fixation pins must be carefully placed, with attention paid to both location and depth of insertion. For thoracolumbar and lumbar fractures, nonsurgical management with a thoracolumbosacral orthosis (TLSO) may be used either in place of or in addition to surgical stabilization (18).Use of Steroids
Various studies of the efficacy of the uses of methylprednisolone in acute SCI were conducted during the 1980s. The National Acute Spinal Cord Injury Study 2 (NACSIS 2) was published in 1990 (19), with the conclusion that patients with acute SCI treated with high- dose methylprednisolone in the first eight hours after injury had better neurologic outcome than did those treated with placebo or naloxone. However, this was an adult study, with only 15% of patients being under 19 years of age and the youngest being 13 years old. Data are lacking in the pediatric population.
Respiratory Function
Most children with SCIs have impairment of normal respiratory function because of their injuries, even in the absence of other trauma causing pulmonary problems. The basic muscles of respiration are the diaphragm, intercostal muscles, abdominal muscles, and neck accessory muscles. Any SCI that weakens one or more of these muscles impairs respiration. The child with weak or absent diaphragm function needs ventilatory support. If the diaphragm is functional but intercostals and abdominal muscles are weak or nonfunctional, the child will need assistance with coughing and may need ventilator support during respiratory illnesses or during sleep.
If the child only has weakness of the abdominal muscles, assistance with coughing may be the only respiratory support needed.All children with acute SCIs should have respiratory function evaluated. At the very least this evaluation should include chest radiographs and measurement of oxygen saturation and end tidal carbon dioxide or arterial blood gases. If the child is able, vital capacity and inspiratory and expiratory forces should be measured on a daily basis until the child is medically stable. Because the child with SCI has restrictive respiratory dysfunction (so-called bellows failure), not lung disease, the earliest pulmonary abnormality will be hypercarbia, not hypoxia. End tidal carbon dioxide measurement is a simple noninvasive way to follow this, and may be used for outpatients as well as for inpatients.
Urinary Function
Most children with acute SCIs have neurogenic bladders. These are initially in spinal shock or flaccid, and may subsequently become spastic or dyssynergic. Flaccid bladders need to be drained either continuously or intermittently. Because indwelling catheters are associated with infections, the child should be converted to a clean intermittent catheterization program as soon as there is no medical reason to have continuous monitoring of urine output.
Gastrointestinal Function
After acute SCI, the gastrointestinal tract usually stops functioning initially, thus requiring the use of nasogastric suctioning. Once the ileus is resolved and the child is taking enteral feeding, a bowel program should be instituted, with the ultimate goal of continence without impaction. The consistency of the stool is normalized through the use of fluids, fiber, and medications, as needed. Evacuation is assisted through the use of digital stimulation or oral or rectal medications.
Fluids and Nutrition
Careful attention must be paid to fluid balance and nutrition in the child with an acute SCI. There must be a balance between enough fluids for hydration and to prevent constipation and not so much that intermittent catheterization must occur too frequently to prevent the bladder from becoming overdistended.
To promote healing, the child must also receive adequate nutrition. A common standard is to start some form of nutrition within 24 hours of injury. Typically, this is parenteral nutrition initially, followed by either oral or tube feedings when the ileus is resolved. For some children who have the ability to eat orally, refusal to eat may be the only way they have of refusing treatment, so nutritional intake should be closely monitored during the acute and rehabilitation hospitalizations.
Rehabilitation
Rehabilitation of the child with spinal cord injury is a lifelong process that starts soon after injury. It does not start and end with admission to and discharge from a rehabilitation unit, and this must be made clear to the patient and family. Goals of rehabilitation will be dependent upon a number of factors, primarily the patient's age, level of injury, and amount of neurologic recovery. Rehabilitation of the child with SCI is comparable to rehabilitation of the child with any other acute change in function, usually with less need for cognitive rehabilitation. The entire rehabilitation process should focus on the whole child in the context of his or her family and community, and be performed by a rehabilitation team of professionals that focuses on the needs of children. It should be noted that on occasion, some older children may be more appropriate for an adult rehabilitation service, while some young adults or people with cognitive impairment may be better served on a pediatric rehabilitation service. When older children are treated on an adult service, it is important that the appropriate pediatric, social, and education services be available.
Goals for rehabilitation should include maintenance or attainment of good health and prevention of secondary complications, while promoting maximal and age-appropriate functional independence. Focus of rehabilitation can range from primarily family education (eg, C4 or higher complete tetraplegia with ventilator dependence) to primarily complete patient functional independence (eg, T10 complete paraplegia).
The goals of rehabilitation will change as the child matures. Table 11.1 lists expected functional goals for levels of spinal cord injury.Mobility
Mobility for the child with SCI begins with the process of learning to sit again. Sitting is compromised as both a result of the lack of neurologic control of the trunk related to the SCI, impairments in the autonomic nervous system, and physiologic adaptations to deconditioning during the acute hospitalization. Hypotension and syncope can result. Support hose, wrapping of the lower extremities with elastic bandages, and abdominal binders may help maintain blood pressure. Progression to sitting is a gradual process and should be started as soon as possible to minimize deconditioning. Short periods of sitting as tolerated can be done multiple times during the day, gradually increasing the duration of the time up. Early use of a power or manual wheelchair (as appropriate) is encouraged. It is important to monitor insensate skin as the duration of time up increases to minimize the development of pressure ulcers. Patients will begin working on bed mobility, rolling in bed, and transferring from the bed to the wheelchair. As the patient improves, more advanced transfers will be worked on.
Standing and walking, either with orthoses or independently, will be done as appropriate. Table 11.2 shows mobility guidelines from recommendations by
| Mobility Guidelines | |||
| LEVEL OF INJURY | AGE | GOALS | ORTHOTIC OPTIONS |
| C1-4 | Bracing available from age 1 year-prepuberty No standing after puberty | Standing | Prone and supine standers (stationary standers) |
| C4-7 | Encourage from ages 1-5 years Available from age 5 years- prepuberty | Static standing and mobility | As above plus parapodiums/ swivelwalkers/mobile stamders |
| T1-5 | Encourage ages 1-10 years after rehabilitation goals are met increase upper extremity strength/endurance); if surgery is performed, intensive gait training available postoperatively | Standing and household ambulation | As above plus RGO |
| Ages 11-21 years need to meet criteria: 6 parallel bar pushups; 25 wheelchair pushups; transfer level heights; and leisure activities. For young children, teaching these activities may need to be incorporated into games and play activities. Children with high tetraplegia may not be expected to manage their own self-care needs, but should be taught how to direct caregivers to perform various activities.Cognition It is important to assess cognition during the rehabilitation of the child with SCI. Just as MVCs are the primary cause of SCI, they are also the primary cause of traumatic brain injury (TBI). Any force significant enough to cause a spinal cord injury can also cause a TBI. Any child who has had an SCI should also be at least screened for a TBI. These screenings may also be useful in assessing possible hypoxic injury in ventilator-dependent children (20). Bladder Management After SCI, most patients develop a neurogenic bladder. While still in spinal shock, this tends to be a hypotonic bladder, but as spinal shock resolves, the bladder often transitions to a spastic bladder. Cauda equina syndrome and damage to the conus medullaris may result in a flaccid/hypotonic bladder. In the acute period, an indwelling catheter is typically placed to drain the bladder. This protects the bladder and kidneys, and allows close management of fluid status. While easy to manage, long-term use of indwelling catheters may lead to increased risk of urinary tract infection, shrinking of the bladder, stretching of the sphincters, and breakdown of the urethra. The goal of bladder management is to gain continence of the urinary bladder, promote independence, minimize urinary tract infections, and protect the kidneys. Voiding pressures need to be maintained less than 40 cm H20 to minimize the risk of ureteral reflux. During the acute phase, baseline evaluations of renal and urinary function need to be performed. These include blood urea nitrogen levels, serum creatinine, urinalysis, urine culture, and renal ultrasound or intravenous pyelogram. When the patient is out of spinal shock, they should undergo urodynamic testing. Clean intermittent catheterization or intermittent self-catheterization is the method most commonly used today for bladder management after SCI. Numerous studies have shown its efficacy and safety for long-term management of the neurogenic bladder. Self-catheterization is easier for males and more problematic for females. Mirrors are frequently used by females to better visualize the urethral opening.For those who cannot independently manage intermittent self-catheterization, external sphincterectomy may be considered for continuously draining the bladder, but this is rarely recommended in children because it destroys any chance for urinary continence when the child is older. It is also rarely recommended in females, as there is no good external collecting device. Condom catheters are commonly used in males after sphincterectomy. Complications of external sphincterectomy may include penile erosions from the condom catheter, need for reoperation, and erectile dysfunction. A surgical procedure such as the Mitrofanoff procedure may be used to ease self-catheterization. This creates a stoma in the abdominal wall, typically through the umbilicus, which allows easier accessibility for catheterization. This is a major surgical procedure and should be performed by an experienced pediatric urologist. It should not be performed during the initial rehabilitation period, but later, after the child has had an opportunity to live at home. Reports of outcomes of this procedure have come from Shriners Hospitals for Children (21,22) and report relatively high satisfaction with the procedure and improved level of independence. Various medications have been used in the management of the neurogenic bladder. In addition to treating urinary tract infections, antibiotics are sometimes used for prophylaxis with a catheterization program or treating asymptomatic bacteriuria. Recently, Clarke et al (23) completed a randomized trial of prophylactic antibiotics in 85 children with neurogenic bladder. They noted a six times higher incidence of urinary tract infection (UTI) in subjects treated with antibiotic prophylaxis compared to those without antibiotics. This was thought to be a result of bacteria developing antibiotic resistance. Schlager's group (24) investigated the use of nitrofurantoin to clear asymptomatic bacteriuria. Approximately 70% of subjects had asymptomatic bacteriuria, which was not cleared by nitrofurantoin. While there was a change in type of bacteria, it resulted in the growth of resistant organisms. At this time, it is not clear that antibiotics should routinely be used for neurogenic bladder, and use may increase the risk for resistant organisms. Cranberry juice is commonly recommended to prevent urinary tract infections, though it has not been shown to be effective in children (25). Anticholinergic agents are commonly used to relax the urinary bladder, which results in a larger bladder capacity and decreased bladder pressures. Commonly used oral agents are oxybutynin, tolterodine, imip- ramine, and hyoscyamine. Side effects include dry mouth, decreased sweating, blurred vision, heat intolerance, and constipation. As children with spinal cord injuries, especially cervical levels, may have impaired thermal regulation, special caution must be used regarding anticholinergics and hot environments. Oxybutynin has been used intravesically to relax the bladder directly and avoid systemic side effects. The tablet is crushed, suspended in distilled water, and instilled in the bladder after catheterization. This practice is particularly useful where environmental temperatures are high and children wish to pursue outdoor activities. In recent years, botulinum toxin A has been used as an intravesicular injection to decrease bladder tone. This was initially evaluated in 2000 and has been increasingly used in Europe, less so in the United States (26,27). Injections seem to last, on average, 9-11 months and are effective with repeat injections (28). Botulinum toxin type A may also be used to relax the external urinary sphincter in a dyssynergic bladder (29). Neurogenic Bowel With the loss of neural control, the gastrointestinal tract loses voluntary control, and peristalsis slows. Stiens and associates reviewed the anatomy, physiology, and management of the neurogenic bowel. A program to control incontinence while preventing impaction must fit into the child's daily life. Factors to consider are premorbid bowel function, timing, consistency, frequency, and volume of bowel movements. The new bowel regimen should duplicate, as closely as possible, the premorbid patterns. If possible, bowel movements should be timed shortly after a meal to take advantage of the gastrocolic reflex. It is often more practical to try to time this after the evening meal, as the child is likely to be home and have more time to manage the bowel movement. Factors to be considered in the new program are diet, physical activity, equipment, oral and rectal medications, and scheduling. The diet should contain adequate fluid and fiber to provide sufficient bulk to facilitate transit through the gastrointestinal tract. Table 11.3 summarizes commonly used medications for bowel programs in SCI. Young children may only need digital stimulation or no special program to evacuate completely. Older children may, likewise, need only digital rectal stimulation to evacuate completely, but, more commonly, one or more oral or rectal medications are necessary. Sometimes, bowel continence cannot be attained just with medications, and surgical intervention may be necessary, especially for those prone to constipation or impaction. The Malone procedure or antegrade continence enema (ACE) creates a stoma to allow antegrade use of enemas to improve bowel evacuation. This procedure has been shown to be effective in improving continence in SCI (30).
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