A Primary Care Provider’s Guide to Pediatric Spinal Cord Injuries Free

Zoe is a 3-year-old female who sustained a C5 spinal cord injury during birth. She recently began an intermittent catheterization program because of recurrent urinary tract infections (UTIs) and vesicoureteral reflux. Her parents brought Zoe into her pediatrician’s office because of episodes of extreme irritability with facial flushing two to three times a day over the past 2 days along with dark colored and smelly urine and a low-grade fever. On examination, her temperature is 39.3°C, heart rate 120 beats/min; respiratory rate 20 inhalations/min, and blood pressure (BP) 115/76 mm Hg. Her face is flushed. She has goosebumps on her trunk and is very irritable. Her pediatrician suspects that Zoe has a UTI; a catheterized urine sample for urinalysis and culture is obtained, and within 5 minutes the facial flushing recedes and a repeat BP is 90/50 mm Hg. The urinalysis demonstrated greater than 50 white blood cells and positive nitrites and leukocyte esterase. Zoe is prescribed a 10-day course of trimethoprim/sulfamethoxazole pending results of the urine culture. Zoe probably does have a UTI; given the fever and history of reflux, she should begin empirically on an antibiotic pending results of the urine culture. However, Zoe was most likely experiencing autonomic dysreflexia given her symptoms and her blood pressure, which on initial evaluation was at least 20 mm Hg above her normal; normal BP for a 3-year-old female is 91/46 mm Hg. Ideally, her parents and pediatrician would have established her baseline BP.

Spinal cord injury (SCI) in youth presents with unique manifestations and complications as compared to adult-onset SCI.^1–3  Similar to caring for all children and adolescents, the primary care clinician must consider the impact of the physical, physiological, cognitive, and psychological changes transpiring during childhood and adolescence in youth with SCI. For example, when evaluating a child with potential autonomic dysreflexia (AD) or orthostatic hypotension, the primary care clinician needs to be cognizant of the blood pressure norms in youth that vary with age. Similarly for prevention of pressure injuries and bladder management, the primary care clinician needs to take into account the changing cognitive issues including communication, executive functioning, and self-management skills. The purpose of this article is to provide a broad overview of pediatric-onset SCI for the primary care clinician. For more in-depth discussions, readers are referred to recent books and book chapters.^1–3  The International SCI Data Sets are useful tools for documentation and monitoring of individuals with SCI.⁴  In caring for youth with SCI, the primary care clinician should utilize the International SCI Core Data Set that is applicable to pediatric SCI. In addition, several pediatric-specific International SCI Data Sets for activity and participation, education, and bowel function have been developed, and they would be appropriate for primary care clinicians to use.^5–7 

In caring for children and adolescents with SCI, the primary care physician should follow the American Academy of Pediatrics’ (AAP) Recommendations for Preventive Pediatric Health (https://www.aap.org/en-us/Documents/periodicity_schedule.pdf).⁸  However, several modifications for SCI are needed.

There is a high incidence of scoliosis and joint contractures in pediatric SCI; therefore, height may be underestimated, and use of arm span that approximates height may be more appropriate.⁹  For youth who cannot stand, obtaining weight may require a wheelchair scale. Challenges of height and weight assessment as well as the loss of muscle mass in paralyzed extremities may impact determination of body mass index (BMI), which is particularly relevant as youth with SCI are at greater risk of metabolic syndrome.¹⁰  Similarly, osteopenia is a common complication, so routine monitoring with dual-energy x-ray absorptiometry (DXA) scans is strongly considered.¹⁰ 

Measurement of BP at all check-ups is essential, especially for youth with T6 or higher SCI as they are at risk of AD.¹¹  BP measurement requires appropriately sized cuffs. Diagnosis is based on the elevation of the youth’s systolic BP, 15 to 20 mm Hg above their baseline. BP is lower in children than adults and tends to be lower in youth with SCI especially those with higher neurological levels. It is imperative to know the baseline BP in youth with SCI rather than use an arbitrary systolic BP of 120 mm Hg to properly identify AD. For example, an 8-year-old child with a C5 SCI may have a baseline systolic BP of 90 mm Hg yet during an episode of AD have a systolic BP of 110 mm Hg, which could be misconstrued as normal using adult standards of a normal BP of 120 mm Hg.

Youth with SCI should receive all recommended immunizations per the Centers for Disease Control and Prevention.¹²  All youth with SCI have compromised pulmonary function and are at high risk of pulmonary infections, particularly those with cervical or thoracic level lesions. Annual influenza vaccines are critical. Youth are also at high risk of invasive pneumococcal disease because of their compromised pulmonary status, and they should receive 13-valent pneumococcal conjugate vaccine (PCV13) and 23-valent pneumococcal polysaccharide vaccine (PPSV23). In addition to the four doses of PCV13 administered from 2 to 15 months of age, a dose of PPSV23 is administered at 24 months of age and at least 8 weeks after last dose of PCV13 and a second dose of PPSV23 is administered 5 years later.

Youth with SCI are at a greater risk of metabolic syndrome and cardiovascular complications than the general pediatric population.¹⁰  Risk factors for metabolic syndrome such as weight gain and lipid profiles should be monitored. Use of BMI may underestimate the degree of obesity in youth with SCI, so alternative measures of adiposity such as DXA scans should be considered. To reduce the risk of cardiovascular complications for youth with SCI, they should receive dietary counseling. Furthermore, the activity level of youth with SCI must be increased, so the primary care provider should coach the youth and their caregivers in the following ways: (1) Youth should be participating in all of the wellness and fitness programs that are offered to other children, including play on the playground, physical education class, and so on. (2) Encourage youth with SCI to participate in community activities similar to children without SCI or to the youth’s peers. (3) Integrate movement and exercise into everyday routines (any movement is better than no movement). (4) Make sure youth with SCI are out of bed every day, including weekends.

Similar for all youth, the primary care provider should provide youth with an SCI and their parent/caregiver with anticipatory guidance, including issues such as use of appropriate automobile restraints, normative adolescent risk-taking behaviors, and preparation for transition to adult care.¹³  Youth should be encouraged to do household chores, participate in extracurricular activities, and plan for employment and career opportunities. Specifically related to SCI, anticipatory guidance should incorporate issues such as shifts from walking to full-time wheeled mobility, decreased adherence with self-care during adolescence, risk for musculoskeletal issues such as fragility fractures and scoliosis, and greater risk for bullying. Moreover, families need to be advised about pubertal changes and sexuality issues, including general and SCI-specific information such as sexual functioning, fertility, and contraception.

Fever is a very common problem for youth with SCI due to general (eg, streptococcal pharyngitis) and SCI-specific causes.¹⁴  Due to the SCI and associated loss of sensation, youth may not complain of dysuria with UTI, abdominal pain with appendicitis, or extremity pain associated with a long bone fracture. Acute infections generally cause most fevers, with respiratory and genitourinary infections being most common. Febrile episodes may be due to noninfectious processes including venous thromboembolism, heterotopic ossification (HO), pathological fractures, or drug-related issues. Youth with tetraplegia or upper thoracic paraplegia may exhibit thermoregulatory abnormalities that may present with hyperthermia or hypothermia because of the inability to vasodilate and sweat in a hot environment or vasoconstrict and shiver in a cold environment. Evaluation must include a thorough history and physical examination to identify general problems such as otitis media or pneumonia as well as SCI-specific concerns, such as a swollen extremity with limited range consistent with fractures, HO, osteomyelitis or septic arthritis, or a swollen scrotum due to epididymitis. Choice of laboratory and imaging studies should be based on clinical findings and may include a urine analysis and culture, a complete blood count with differential, erythrocyte sedimentation rate, and C-reactive protein. Serum amylase and lipase, plain abdominal radiographs, abdominal and pelvic ultrasound, and computer tomography may be helpful in evaluating the patient for potential intraabdominal disorders.

Youth with T6 or higher SCI lose supraspinal control of the spinal autonomic circuits.¹¹  As a result, they are susceptible to AD, which occurs when there is a noxious stimulus below the neurologic level such as a distended bladder, stool impaction, or a pressure injury. Adolescent females may experience AD during menstruation because of menstrual cramps or use of tampons. Noxious stimuli result in increased sympathetic output and vasoconstriction below the neurologic level, which causes hypertension. In response, vagal tone increases and may cause bradycardia. There is decreased sympathetic output with vasodilation that occurs above the neurologic level resulting in flushing of the face; however, the vasodilatation above the neurologic level does not compensate for the extensive vasoconstriction below the zone of injury so hypertension persists, resulting in a pounding headache. Symptoms of AD include hypertension, flushed face, pounding headache, piloerection, and bradycardia; although bradycardia seems to be less common in youth. Degree of hypertension can be variable depending upon the youth’s age, with elevation of the systolic BP of a least 15 mm Hg in children and 20 mm Hg in adolescents. As BP varies with age and gender as well as level of SCI, it is important to establish baseline BP as the youth grows.¹⁵  There can be extreme elevations of BP resulting in severe and life-threatening complications such as myocardial infarct, intracranial hemorrhages, and seizures. It is crucial to know the youth’s baseline BP in order to identify a BP elevation consistent with AD (Table 1).

In view of the varying cognitive and verbal communication abilities of children, symptoms of AD may not be expressed or may be communicated less clearly. For example, in preschool-aged children, AD may present with vague symptoms rather than expressed complaints of a pounding headache. Medical alert identification should be utilized and appropriate education on symptom recognition and emergency management must be provided for those adults who are significantly involved in the lives of children with SCI, such as teachers, school nurses, coaches, and community-based health care providers.

Symptomatic measures are generally successful in managing the majority of episodes of AD. These include elevating the head and lowering the legs and inspection for any obvious constricting clothing. Catheterize the bladder for those on an intermittent catheterization program after installation of lidocaine lubricant, if possible. For those with an indwelling catheter (Foley or suprapubic), the functioning of the catheter needs to be assessed to exclude kinking of the catheter or tubing and a full urine collection bag should be emptied; replacement of the indwelling catheter or flushing of the catheter may also be indicated. If these measures do not resolve the AD, assess and manage fecal impaction, which should be conducted by an experienced caregiver or health care provider because of the risk of exacerbating the hypertension. Management of an episode of AD with systolic BPs above 150 mm Hg or not responsive to conservative measures includes either topically applied Nitropaste (0.5 to 2 inches) or nifedipine (0.25 mg/kg or 10 mg in adolescents weighing 40 or more kg) administered by chew and swallow for those who can follow directions or sublingually for younger children and infants. Referral to an emergency department of a child experiencing AD should be considered if the episode persists despite symptomatic management including catheterization or when the systolic BP remains above 150 mm Hg.

Hyperhidrosis is seen primarily in individuals with tetraplegia or upper thoracic paraplegia.¹¹  Stimuli that may incite hyperhidrosis include UTI, urolithiasis, posttraumatic syringomyelia, and tethering of the spinal cord, or it may be unexplained. Treatment should be initiated if it is distressing to the youth, impairs function, or increases risk of developing pressure injuries. Management should begin with avoidance and alleviation of precipitating factors. Medications that inhibit sympathetic overactivity, such as propantheline or transdermal scopolamine, can be used.

Orthostatic hypotension (OH) is not an uncommon issue for youth with SCI and may result in light-headedness or syncope.¹¹  Conservative management to prevent symptomatic OH may include graduated elastic stockings, abdominal binders, adequate hydration, and gradual assumption of an upright position. Potential treatments for OH not responsive to conservative measures include midodrine, pseudoephedrine, and fludrocortisone.

When a youth presents with a swollen extremity, a careful history should be obtained to elicit a potential traumatic event or a febrile illness. Examination should identify the site of the swelling, the presence of warmth, and the range of motion of adjacent joints. Potential traumatic etiologies include fractures, most commonly supracondylar, intramuscular hematomas, or acute dislocation of the hip. Infectious causes include osteomyelitis or septic arthritis. Other potential causes include HO most commonly involving the hip or a deep venous thrombosis.

Spine deformities are an extremely common problem in pediatric SCI, especially if the injury is sustained prior to skeletal maturity; 98% will develop scoliosis and 67% will require surgery.¹⁶  Risk of scoliosis is reduced to 20% with approximately 5% requiring surgical correction, if injury occurs after skeletal maturity. Radiographs of the entire spine should be obtained every 6 months prior to puberty and every 12 months thereafter. Prophylactic bracing with thoracolumbosacral orthoses may be effective in delaying the need for spine surgery, but challenges include poor adherence and interference with mobility and independent functioning.

Hip subluxation, dislocation, and contractures are frequent complications in children with SCI, especially if injured at younger ages, with hip instability being observed in 100% of children who were injured when they were less than 5 years of age.¹⁷ 

Pressure injuries are one of the most common complications for youth with SCI.^18,19  Toddlers and younger children may be at risk because of inadvertent trauma from activities and play typical of these age groups. Preventive interventions should be developmentally based, with responsibility gradually shifting from parents to the youth. For example, a timer on a watch or phone may be a helpful reminder for pressure relief. As children grow, new equipment must be matched to their size. Properly fitting wheelchairs and adequate cushions must be prescribed, and pressure mapping should be performed. Main principles of managing a pressure injury are to remove pressure and maintain a clean and moist wound base.

Many youth with SCI demonstrate spasticity. Increased tone may be used functionally by some youth; however, when problematic, attention should be directed to potential inciting factors, such as noxious stimuli below the zone of injury (eg, ingrown toenail or hip dislocation).²⁰  Management goals are to improve function, prevent complications, and alleviate pain; these goals include prevention, nonpharmacological interventions (eg, relief of inciting factors, stretching and range-of-motion exercises, positioning), medications, and invasive procedures. Medications should be considered when spasticity affects functioning and is unresponsive to conservative treatment. Baclofen administered orally is the initial medication of choice. Other medications include diazepam, clonidine, dantrolene, gabapentin, and tizanidine. For spasticity that does not respond to standard management, options include intrathecal baclofen, selective dorsal root rhizotomies, epidural spinal cord stimulation, and localized injection of botulinum toxin.

The expectation is that youth with SCI should be continent and independent in their bladder management. Intermittent catheterization via a clean technique is the standard management of neurogenic bladder in youth with SCI.²¹  Intermittent catheterization is generally initiated when the child is approximately 3 years old or earlier if the child is experiencing recurrent UTIs or exhibiting renal compromise. Children with adequate hand function should be taught how to perform self-catheterization when they are 5 to 7 years old. Screening for and treatment of asymptomatic bacteriuria is not recommended, and prophylactic antibiotics should not routinely be used.²²  Treatment should be limited to symptomatic UTIs, as manifested by systemic toxicity (such as fever, chills, AD, or exacerbation of spasticity).^22,23  It should be noted that the Clinical Practice Guidelines for the Management of Asymptomatic Bacteriuria: 2019 Update by the Infectious Diseases Society of America stated that “further studies in SCI patients managed with intermittent or indwelling catheterization are needed to evaluate the significance of nonspecific symptoms, including incontinence and cloudy and malodorous urine.”²² 

Management of incontinence may include anticholinergics, modification of fluid intake and catheterization schedule, intravesical injection of botulinum, and treatment of urologic complications such as UTI and urolithiasis. Urodynamics should be performed in patients with persistent incontinence. Some youth with limited bladder capacity may require a bladder augmentation. A continent catheterizable conduit, known as the Mitrofanoff procedure, consists of creating a catheterizable conduit using the appendix or a segment of small bowel, which connects the bladder to a stoma in the umbilicus. Using a continent catheterizable conduit, youth with limited hand function, such as those with C6 or C7 injuries, are able to self-catheterize.

Goals for bowel management in youth with SCI include complete and regular emptying, continence, short duration of the bowel program, aesthetics, and prevention of complications.²¹  Bowel programs often conflict with youth’s schedules and interests. Constipation is a very frequent complication, which not uncommonly results in AD and/or emergency room visits. Patient and parent education regarding bowel management and prevention of constipation should be provided at all routine visits.

Bowel programs are initiated when children are 2 to 4 years of age. Fundamental aspects include independence, privacy, and regularity (frequency and time of day). Bowel programs should take place on a toilet or a commode with the youth sitting as it facilitates defecation. Other components may include oral laxatives (eg, sennosides, polyethylene glycol), stool softeners (eg, docusate), and suppositories (eg, docusate/Enemeez, bisacodyl/Magic bullets). An option for youth who are unsuccessful with standard bowel program interventions is the Malone antegrade continence enema (ACE procedure). With the ACE, antegrade evacuation of the bowel is accomplished by administering an enema directly into the cecum. This is delivered into the cecum via the appendix, which is accessible through an abdominal wall stoma.

Youth with SCI are at risk of latex allergy, immediate-type, immunoglobulin E (IgE) mediated, reactions.²⁴  Latex allergy results from frequent and extensive contact with latex, young age of initial exposure, and longer duration of exposure to latex. Reactions can occur by direct contact via cutaneous, mucosal, intravenous, or serosal routes or by airborne dissemination of latex antigens that have adhered to glove powder. Latex allergy may manifest as localized or generalized urticaria, wheezing, angioedema, or anaphylaxis. Diagnosis is made by a consistent history or utilizing in vitro assays. Clinical manifestations may be subtle (eg, a blotchy facial rash when playing with a balloon containing latex). Latex allergy should be suspected in youth who experience unexplained intraoperative allergic reactions or those with allergies to bananas, kiwi, avocados, or chestnuts. Youth with SCI should be cared for in a latex-free environment, wear medical alert identification, and carry auto-injectable epinephrine. Youth, family, and those working with the youth must be educated about the latex allergy.

Respiratory problems may affect youth with any level of SCI, especially those with cervical and thoracic level injuries.¹⁴  Severity is a function of the neurological level of the SCI. Individuals with injuries at C2 to C4 have significant respiratory compromise because of impairment of the diaphragm, intercostals, and abdominal muscles. Those with C2 to C3 injuries generally require chronic ventilatory support. Respiratory compromise in those with lower cervical and high thoracic injuries (C5-T4) is a result of intercostal weakness and absence of abdominal musculature. Youth who have mid to lower thoracic injuries (T5-12) may still experience some degree of respiratory dysfunction, such as a weak cough, due to abdominal musculature involvement. Youth with tetraplegia are at risk of sleep-disordered breathing manifested by sleep problems, restlessness, confusion, cognitive changes, daytime sleepiness, or weight loss. Those with ineffective cough may benefit from cough assistance, which may be accomplished using manual cough assistance (quad cough) or a mechanical device such as the mechanical insufflator/exsufflator (cough assist).

Pain is a significant problem for youth with SCI and may be disabling and negatively affect school, work, and social interactions.²⁵  Pain may be nociceptive or neuropathic or a combination. Neuropathic pain may be radicular, originating from the area of trauma due to compression of a nerve root, or may represent central pain or dysesthesia. Evaluation of pain in infants and younger children is complicated by their developmental level communication abilities (Table 2).

Management consists of physical modalities, psychological interventions, and medications. Physical modalities may include physical therapy, hydrotherapy, and transcutaneous electrical neural stimulation. Psychological interventions may include cognitive behavioral therapy, including relaxation, activity pacing, and cognitive restructuring. Medications include antidepressants such as amitriptyline and gabapentin. Other medications that may be beneficial include carbamazepine, clonidine, and phenytoin.

Sexuality is frequently overlooked when caring for youth with SCI.^26,27  General and SCI-specific issues should be addressed with youth and families. Youth and parents must be educated in a prospective and optimistic manner about future sexuality issues, including romantic relationships and fertility. Sexuality education should be addressed directly to older children and adolescents without their parents being present. Females who have sustained SCI and their parents should be reassured that the SCI will result in minor or no abnormalities in fertility and no delays in onset or resumption of menstruation.

Education is an important part of the lives of youth with SCI. Youth should return to school as soon as possible after injury, and ideally they should return to the school that they had previously attended prior to injury.²⁸  Returning to school allows youth with SCI to reestablish friendships and peer interactions. Additionally, education is a major determinant of adult employment. Returning to school can be a traumatic event for the patient, fellow students, and the teachers. Transition back into school can be significantly improved by having the patient visit his school prior to discharge from inpatient rehabilitation. A 504 plan or individualized education plan (IEP) may be needed to address environment accommodations and academic supports.

SCI has a wide-reaching impact on youth with SCI and their family.²⁷  While most are resilient and adjust well to life after injury, youth face significant challenges in that they must cope with SCI while also navigating the tumultuous changes and complexities of childhood, adolescence, and young adulthood. Adolescence may be particularly challenging due to the concurrent psychosocial and physical processes inherent in this time period. For example, adolescents will manifest greater dependence on caregivers during a time when autonomy is expected to increase. Psychological evaluation and interventions should be responsive to the changing developmental needs as well as SCI-related factors. Psychological support and interventions must also be provided for the parents/caregivers, siblings, and other significant family members because of the significant impact an SCI has on the family. The well-being of parents is an integral part of pediatric SCI rehabilitation. Support and peer groups are beneficial for patients, parents, and other family members. To minimize burnout in parents, respite care is essential, particularly if their child has high tetraplegia with complicated and intense needs.

Substance abuse may contribute to the onset of the SCI as well as affect outcomes.²⁹  Moreover, long-term substance use can increase the risk of or worsen secondary health conditions, such as pressure injuries, UTI, and depression. Youth with SCI may seek substance use as a method of coping or as part of normative experimentation of adolescence. Clinicians should counsel youth and their families regarding the use of substances and its potential associations with future outcomes.

The primary care provider along with youth with SCI and their family constitute the “medical home,” which is the most critical and central player in the overall SCI management. The primary care provider must help orchestrate the multitude of health care providers in managing the youth with SCI and in so doing must ensure that the goal of a full and productive life remains the guiding principle.

Dr. Zebracki is Co-Editor and Dr. Vogel is former Co-Editor of Topics in Spinal Cord Injury Rehabilitation. The other authors report no conflicts of interest.