Pediatric Rehabilitation-Focused Review on Pediatric Spinal Cord Disorders through the AAPMNR. This review was originally developed in 2017 by Dr. Angela Senner of Gillette Children's Specialty Healthcare. It has since been updated and expanded by myself, Dr. Abigail Case at Children's Hospital of Philadelphia. The learning objectives of this review will be for participants to be able to describe the epidemiology and etiology of acquired spinal cord injury, identifying common complications and treatment strategies. Participants will also recognize common characteristics of pediatric acute transverse myelitis. Acquired Spinal Cord Injury Fortunately, pediatric spinal cord injury is a relatively rare event. The overall incidence of pediatric spinal cord injury is approximately two cases per 100,000. In the United States, this estimates to be 1,450 patients admitted each year for acute spinal cord injury. It's important to note there are several differences between adult injury and pediatric injury. In general, pediatric spinal cord injury has a higher mortality in comparison to adults. This is most likely due to the fact that pediatric patients have higher cervical level injury. There are also gender differences. Similar to adults, pediatric patients have a higher incidence in males than in females. Although there are some studies to suggest in the young zero to five-year-old patients, there's a slightly higher predominance of female patients. Pediatric spinal cord injury can be broken into traumatic and non-traumatic causes. Traumatic causes, by far, are the largest cause of pediatric spinal cord injury. Most commonly are motor vehicle collisions, which account for up to 50% of injury. It's important to note that almost 70% are unrestrained at the time of the accident. Following are falls, sports, and violent etiologies. Violent etiologies have continued to increase in the United States over the past several decades, with gunshots being the most common cause. Additionally, spinal cord injury without radiologic abnormality is a pediatric phenomena in which patients present with an objective sign of myelopathy but have no findings on CT or X-ray studies. Non-traumatic causes can include congenital, inflammatory, neoplastic conditions, infectious, vascular abnormalities, toxins, and radiation. Injuring the pediatric patient's spine takes significantly less force than injuring an adult's spine. This is related to anatomical and growth pattern differences in pediatric patients. The pediatric head circumference reaches 50% of the adult head size by age 2. This leads to a higher fulcrum, which can lead to cervical injury more easily than in adult patients. In addition, the pediatric vertebral column is much more tolerant to stress and strain than the spinal cord is, which leads to spinal cord injury in pediatric patients. There are three types of severe forces that cause spinal vertebral injuries. Longitudinal compression, which crushes one vertebra lengthwise against another. An example of this is a patient who dives into shallow water and hits their head on a hard surface. Hinging, which results when the spinal column is subjected to sudden extreme bending. An example of this is when a patient falls forward and hits their chin on a hard surface, which then hyperextends the neck and can lead to a hinging injury. Shearing combines hinging and twisting, or rotation. And often, this occurs in an example where a pedestrian is hit by a motor vehicle and the individual is spun around. As mentioned before, spinal cord injury without radiologic abnormality is unique to pediatric patients. It accounts for up to 30% to 40% of all pediatric spinal injuries and is very uncommon in adults. This can be explained because of the ligamentous structure differences in children and that they're more elastic than their osseous structures are immature. When a patient presents with definitive signs of myelopathy without any objective findings on CT scan or x-ray, they most likely need an MRI to rule out any abnormalities. In general, pediatric spinal cord injury without radiologic abnormality involves the cervical spine. It occurs with greater severity in children under the age of eight years. It is also important to note that there's a wide variety in presentations. They can present as a subtle transient neurologic deficit, similar to a concussive type syndrome with paraseasis and weakness that have full recovery, to a definitive injury with little room for improvement. In addition, patients may have a delay of presentation of symptoms for up to 48 hours after injury. This is important to monitor these patients closely. As mentioned previously, there are several non-traumatic causes that lead to pediatric spinal cord injury. Listed below are several pediatric conditions that can predispose. Down syndrome or trisomy 21 can have up to approximately 15% of edulino-axial instability leading to cervical injury. Achondroplasia, clipophile syndrome, which is a congenital fusion of variable numbers of the cervical vertebra, associated with deficits including scoliosis, renal anomalies, and an elevated scapula. Morquio syndrome or a mucopolysaccharidosis type 4, which is associated with hypoplasia of the odontoid process. And Larson syndrome, characterized by multiple joint dislocations, a flat facies, and short fingernails. This may also have associated cervical vertebra hypoplasia. Spinal shock is an important component of acute spinal cord injury. It is a transient period of total flaccid paralysis of all skeletal muscles and loss of spinal reflexes below the level of lesion and the acute injury. It may last for variable periods of time, from several hours to several weeks, on average, resulting in 4 to 12 weeks. Reflexes begin to return after 24 hours. The return of the bulbous spongiosis reflex denotes the end of spinal shock. It's important to distinguish spinal shock from neurogenic shock, which is a type of distributive shock that results in sudden loss of sympathetic tone, which results in hypotension and bradycardia and needs to be treated with ionotropes or chronotropes. Pediatric American Spinal Injury Association Examination. Scoring and classification are similar to adult patients. However, there are many modifications made for children in administering this examination. First, modifying instructions to make things more understandable and fun for children. We also involve parents, asking parents what terms they use for sensitive information. Asking parents what terms they use for sensitive areas of their bodies. Patients may often benefit from adult or parent demonstration. It's important to note that there is higher inter-rater reliability in children greater than six years of age, which correlates with the developmental stage in which they are able to understand more appropriately. As much as it is important to do an anorectal examination to determine the completeness of injury, this is much less reliable in younger children. Respiratory function is often impaired during acute spinal cord injury. The higher level of lesion, the more affected function is. All parameters in respiratory function tend to decrease, including total lung capacity, vital capacity, expiratory reserve volume, and functional residual volume. Residual volume is the only parameter that increases. At the highest cervical level, vital capacity is approximately 5% to 10%, with the cough being absent. Mid-lower to cervical, vital capacity is 20%, and the cough is weak and ineffective. At the high thoracic level, vital capacity is 30% to 50%, and the cough is weak. Injuries at the lower thoracic and below, respiratory dysfunction can be minimal, and vital capacity is essentially normal with a strong cough. Children with injuries at the C5 level and below, despite having full use of their diagram, may still struggle with respiration in the acute phase of their spinal cord injury, for the reasons mentioned on the previous slide, as well as weakness and deconditioning of their secondary respiratory muscle groups. Respiratory strength training is an essential component to their rehabilitation to maximize their residual respiratory function. There are other common compensation strategies, including wearing an abdominal binder, having hyperinflation techniques, a manual cough assist, which includes a thumb under the diaphragm, which is also called a quad cough, and a mechanical insipilator-exfilator, or a cough lighter cough assist machine. Neurogenic bladder is a chronic health concern for patients of pediatric spinal cord injury. The primary goals of bladder management are to preserve renal function, prevent life-threatening complications, and promote continence. These are extremely important for pediatric patients, as they are injured earlier in life and require a longer time to acquire renal damage. In line with other pediatric rehab management, it should be delivered in an age-appropriate manner. Intermittent catheterization should be introduced at three years of age, with the goal to complete independence by five to seven years. The formula to calculate pediatric bladder size is relatively simple. Patients age in years plus two, and then multiplied by 30 to get to milliliters, or cc's. Urology and PM&R work closely together to minimize secondary complications related to neurogenic bladder. Neurogenic bowel is another chronic problem for pediatric patients of spinal cord injury. The colonic transit time is almost doubled in injury, going from an average of 39 hours for an intact bowel to 80.7 plus or minus 11 hours for total transit time. It's important to distinguish between an upper motor neuron and a lower motor neuron injury when considering a neurogenic bowel management. Bowel programs can be successfully introduced around two to four years. Most times, you want to trial a conservative measure first. This includes taking advantage of the gastrocolic reflux, using gravity-aided postures, and applying suprapubic pressure, also known as the Cradet maneuver. However, unfortunately, more than 80% of children will require the use of oral, rectal, or combination medication. Hypercoagulopathy is an important complication of acute spinal cord injury. Patients with spinal cord injury are at an increased risk of deep venous thrombosis and pulmonary embolism. It's important to know that pre-pubital, younger than age 12, approximately, their DVT risk is low. Chemoprophylaxis is not routinely recommended in this age group unless there are other significant risk factors present. Treatment protocols for DVT are similar to adults, with the exception there's weight-based dosing of low molecular weight heparin, and it requires measurement of an anti-factor 10A level due to the variable rates of metabolism in children. Risk of DVT is greatest within the first 10 days of spinal cord injury, and the highest risk is between 7 to 10 days. Risk continues to decline after that. Bone and metabolic health. Pediatric spinal cord injury patients are four times greater risk of fracture than adults due to decreased bone mineral density. In fact, pathologic fractures are present in approximately 14% of children and adolescents with injury. Distal femur and proximal tibia are the most common sites. Many spinal cord injury patients are also at risk for metabolic syndrome, type 2 diabetes, cardiovascular disease, and stroke. Obesity is primarily thought to be the cause. Hypercalcemia. Hypercalcemia can be a serious complication and can affect around 23% of children, particularly adolescent males. The classic symptoms that you should be aware of are abdominal pain, polyuria, vomiting, generalized malaise, and potential psychosis. It is associated with an increased risk of renal stones and renal failure. Treatment is generally thought to be aggressive hydration, furosemide, calcitonin, and sometimes bisphosphonates are considered. Autonomic dysreflexia is an important part of acute spinal cord injury. As with adults, injuries at the level T6 or higher can result in autonomic system dysfunction. The pathophysiology. Major splanchnic outflow is T6 through L2. Intact sensory nerves below the level of injury transmit noxious afferent impulses to the spinal cord, which ascend in the spinothalamic and posterior columns. Sympathetic neurons and the intermediolateral gray matter are stimulated by these ascending impulses. Sympathetic inhibitory impulses that originate above T6 are blocked due to their injury. Therefore, the injury is relatively unopposed sympathetic outflow. More than half of children with T6 or higher injuries are likely to experience this. Older children have the typical classic symptoms, such as facial flushing, headaches, and pyloerection. Children younger than age five rarely show these signs and symptoms. Another important non-traumatic cause for acquired spinal cord injury is acute transverse myelitis. This is an immune-mediated central nervous system disorder contributing to 20% of children experiencing an acquired demyelinating syndrome. MRI lesions are often centrally located with high T2 signal intensity involving gray and neighboring white matter. Important to note, children generally have a better outcome than adults, with one half making a complete recovery by two years. One of the most important questions you may be asked when taking care of pediatric patients with acute spinal cord injury is, will my child always be paralyzed? It is important to be honest with the child and the family. You must explain the facts of the situation as clearly and truthfully as possible. This depends on how much of the spinal cord has been damaged, and we know that incomplete lesions have a better chance of improvement. Usually, the biggest improvement occurs in the first few months. As more time goes by without improvement, the less likely it is that major improvement will occur. Improvements in initial ASIA score exams are typically within the first three months. An ASIA A who converts to an ASIA B or greater is 66% likely in the first 60 days and the remaining 33% by 90 days. Surgery done in hours to days after injury to release pressure on the spinal cord or nerves can bring back some movement or feeling. These are important facts to keep in mind when discussing these sensitive discussions with families. Spinal cord injury functional outcomes. There are key functional levels that have been determined from the Spinal Cord Injury Consortium database. At the level of C1 to C3, you can anticipate a patient will be ventilator dependent. At C5, they can, with the help of inpatient rehabilitation, feed themselves with adaptive equipment. The level C6 is considered to be the highest level of complete injury consistent with independent living, with use of orthoses and human aid, without human aid. C7 and C8, completely independent from a wheelchair level. L2 and L3, the gait becomes more functional. L3 is considered to be the highest level where functional community ambulation occurs. First question, hypercalcemia after pediatric spinal cord injury. A, it's more likely to occur in females. B, most often occurs six months or more after injury. C, more often occurs in cervical level injury patients. Or D, has specific symptoms of joint pain. Answer, C, more often occurs in cervical level injury patients. Again, hypercalcemia is most likely to occur in adolescent boys and in those with higher level injury. It's most common in the first two to three months after injury. The signs and symptoms are vague, but can include abdominal pain, lethargy, nausea, dehydration, renal stones, osteopenia, and cardiac arrhythmias. It must be treated appropriately with fluids, diuretics, calcitonin, and potentially antibiotics. Second question, a 16-year-old patient with a history of complete tetraplegia C4 level sensory, C5 motor level Asia A, presents with weakness described as unilateral difficulty using motorized wheelchair stick, presents to the ED. No fevers, no respiratory issues, and no autonomic dysreflexia. He is one year out from his injury. He otherwise feels well. What is the most likely cause of his symptoms? A, re-injury, B, progression of disease, C, post-traumatic cystic myelopathy, or D, spinal artery infarct. The answer is C, post-traumatic cystic myelopathy. Again, this can happen at the injury site. The pathophysiology is not totally understood. Onset can be within months, although usually within the first few years. The etiology is thought to be an abnormality in fluid flow, causing collection. It also can have vague symptoms, such as loss of motor or sensory function, increased spasticity, change in neuropathic pain levels, increased autonomic dysreflexia, or a loss of bowel or bladder function. You often need to evaluate this with an MRI. You may need neurosurgical evaluation. In some patients, pain has been the most common presentation and symptom. It can be localized or diffuse, and it's frequently characterized as a dull ache or burning. Other symptoms, again, include increased weakness, numbness, increased spasticity, or hyperhidrosis. Thank you.

pediatric spinal cord disorders

epidemiology

etiology

common complications

treatment strategies