Today we're going to talk about pediatric spinal disorders. I have no relevant disclosures. Today's learning objectives are classification, diagnosis, and treatment of scoliosis, as well as diagnosis and treatment of the following common conditions, kyphosis, Sherman disease, Kleipel-Fell syndrome, congenital muscular torticollis, spondylolithesis and spondylolosis, and herniated vertebral discs. First we're going to discuss scoliosis. Scoliosis is a frontal plane deformity of the spine that is greater than 10 degrees with frequent coexistence of rotational deformity. It is the most common pediatric spinal deformity. It can be classified as congenital, idiopathic, neuromuscular, and functional types. Then idiopathic scoliosis is further divided into subtypes of infantile, juvenile, and adolescent. If untreated, severe scoliosis can lead to respiratory compromise, seating compromise, pain, gait impairment, difficulty with activities of daily living, and physiological distress. Spinal development is a complex process beginning in the first month of gestation when the mesoderm cells surrounding the notochord begin to differentiate into sclerotomes. These will ultimately form vertebral bodies and the arches. Injury and early gestation often affect nearby organs, primarily cardiac, renal, and gastrointestinal systems. Approximately 60% of those with spinal anomalies have other congenital malformations, so screening for these areas are important. Unlike limb growth, vertebral growth is nonlinear. Two major growth spurts typically occur, the first being before age 3 and the second during puberty. Tanner stage and or Risser sign classifications are helpful in predicting growth spurts, the progression of scoliosis, and the cessation of growth. While race, hereditary, physical activity, physical disability, and nutrition may affect growth, growth typically accelerates girls at Tanner stage 2 and in boys at Tanner stage 3. The Risser sign is the use of a Risser line seen by posterior anterior radiographs of the iliac crest assist in staging of skeletal maturity and predicting future growth. The Risser system is based on ossification of the iliac crest proceeding from the lateral to medial and extends from grade 0 where there is no ossification to a grade 5 where you have complete fusion to iliac apophysis. A Riser 1 represents the period of most rapid skeletal growth and correlation of the Riser sign and degree of scoliotic curve can be predictive of curve progression. So here you're seeing two different signs there's the French and US Riser staging systems that have six stages. Stage 0 in the US and French system is defined as no ossification of the apophysis. In the French system ossification of the iliac wing is divided into thirds represent stage 1, 2, and 3. Stage 4 defines the period when the apophysis commences fusion to the iliac wing posteriorly whereas stage 5 is when fusion of the apophysis to the iliac wing is complete. Ossification of the apophysis in the US system is in divided into quarters defining the first four stages 1, 2, 3, 4 where Riser stage 5 describes the period when the ossification apophysis fuses to the iliac wing. For curve classification and naming, scoliosis curves are named by their direction, location, and magnitude. The curve's apex or the most laterally deviated vertebrae from the sacral line indicates its named direction and location and measurements by the Cobb angle provides its most reliable magnitude. If more than one curve exists the largest degree curve is designated as the major and the other as the minor. Curves over 60 degrees are associated with restrictive lung disease. Here you can see a picture showing the different naming so there is a thoracic curvature, we have a double major in the next one, a thoracolumbar, double thoracic, and a lumbar classification. The measurement of the Cobb angle involves estimating the angle between the two tangents of the upper and lower end plates of the upper and lower end vertebrae respectively. The severity definition for scoliosis is determined by using the Cobb angle. The condition of the spine is associated with a spinal curve instead of scoliosis when the Cobb angle is less than 10 degrees. A Cobb angle in the range of 10 to 20 degrees is considered mild scoliosis. A moderate scoliosis is when the Cobb angle ranges from 20 to 40 degrees and a Cobb angle greater than 40 degrees denotes severe scoliosis. The scoliosis exam will vary depending on the patient's age and associated diagnosis. For example in a patient with a spinal cord injury or spina bifida if they have a rapid curve progression think of a syrinx or a tethered cord. So getting the history, a positive family history is pertinent in congenital and idiopathic scoliosis. Presence of back pain may indicate a serious dischitis or tumor. Keep in mind that a rapid curve progression, bowel and bladder changes, recent trauma, associated weight loss, muscle weakness, or joint pain can point to other serious primary processes such as a syrinx or tethered cord, spinal fracture, rheumatological disease, osteoblastoma, or a hip deformity. You want to assess reflexes, strength, range of motion, general posture, and gait. And children with disabilities need to assess seating systems, improper walker or crutch height, and truncal weakness. You also want to assess the skin for cafe au lait spots, webbed neck or low hairline, and hairy patches or skin dimples. So this you could be looking for a neurofibromatosis, clypophile syndrome, and spina bifida occulta. You're going to want to get full spine AP lateral x-rays. May require a CT or MRI for congenital or infantile scoliosis or if you're assessing for a more serious condition. Treatment is going to be between orthotic management and surgery. Orthotic management is not appropriate, effective, or recommended for all forms of scoliosis. Long-term bracing while reducing curve progression and maintaining flexibility needs to be considered carefully with respect of function, social development, and self-esteem. Curves less than 40 degrees are typically compatible with bracing but their location affects brace choice. Curves with the apex at or below T7 are typically managed with a soft or rigid TLSO which allows more functional activity than the Milwaukee brace used for curves above T7. This brace often incorporates a chin and head pad. It's more restrictive and therefore less well tolerated. It is recommended that both braces be worn 16 to 24 hours a day in order to be effective. So compliance is challenging for these braces. Achieving a balanced spine, a solid arthrodesis, and a reduction in deformity are the primary goals for intervention. The surgical techniques may vary with the type of scoliosis. You want to do continuous intraoperative spinal monitoring for prevention of neurological injury during the surgery. So complications can include infection, pseudoarthrosis, anemia, hypotension, and hardwell failure. So the different types of scoliosis. Congenital scoliosis, it accounts for approximately 20% of all scoliosis due to prenatal disruption of the vertebral formation with a hemivertebrae or wedge vertebrae or vertebral segmentation which is a blocked vertebrae or unilateral bar. A single hemivertebrae is the most common anomaly seen. You may have abnormalities of the trachea, esophagus, renal tract, GI tract, lungs, heart, radius, ears, lips, and palate can often accompany congenital scoliosis. Up to 25% of children may have renal disorders, 10% may have cardiac problems, and 30% may have spinal dystrophism. Scoliosis is primary symptom in Bader syndrome and thoracic insufficiency syndrome. Immediate surgically referral is recommended if a congenital spine anomaly is identified. They're going to want to get an MRI of the spine. Typical orthosis are ineffective in congenital scoliosis. Approximately 50% of children with congenital scoliosis require surgical intervention at an early age before spinal rigidity or secondary pulmonary deficiencies occur. Congenital kyphosis is most common at T10 to L1 and is due to failure of vertebral segmentation and or formation. So here's just some pictures showing the different types of congenital scoliosis, whether you have a partial unilateral failure of formation which is the web vertebrae. Next you're going to see a hemivertebrae. The third picture shows a congenital bar where there's a unilateral failure of segmentation of the vertebrae. And then the last picture shows a block vertebrae or bilateral failure of segmentation of the vertebrae. Next is idiopathic scoliosis. More than 80%. Next type is neuromuscular scoliosis. This is common in neuromuscular diseases such as cerebral palsy, muscular dystrophy, and spinal cord injuries. They are benign early and can progress rapidly, usually unresponsive to bracing and may require surgical intervention to slow the progression, can continue to progress even after maturity. Orthosis will not prevent progression of scoliosis but may help with trunk control and sitting posture. And wearing an orthosis will not prevent the need for surgical intervention. After a softer brace will be more tolerable to the patient. Neuromuscular curves over 50 degrees may continue to progress at a rate of 1.5 degrees per year even after skeletal maturity. Despite the challenges of surgical correction in children with neuromuscular scoliosis, studies have shown that curve degree, lung function, seating position, and activities of daily living may all improve post-operatively, potentially improving quality of life and caregiver abilities. Next is functional scoliosis or secondary scoliosis. It is a flexible non-bony curve secondary to leg-length discrepancy, herniated disc, mondial lathesis, dischitis, muscle spasms, trauma, arthritis, or hip disease. Treatment of the underlying problem typically resolves the curve. Next we're going to discuss kyphosis. So kyphosis is the curvature of the spine in the sagittal plane. Thoracic spine normally has a kyphotic curve of 25 to 50 degrees. Excessive kyphosis may be purely postural in nature or may be associated with a number of pathological conditions such as vertebral anomalies, Sherman disease, neuromuscular afflictions, skeletal dysplasia, and metabolic diseases. Kyphosis can also develop following a spinal trauma or surgery. Patients with structural deformity may complain of a backache aggravated by motion. So for postural kyphosis, usually seen in pre-adolescence, it's a flexible thoracic kyphosis that is correctable with hyperextension. Treatment is exercise program to strengthen the trunk and abdominal muscles. In Sherman disease, this is a disorder of unknown etiology, most common cause of a fixed kyphotic deformity. It's unable to correct with hyperextension. It's stiff. So lateral radiographs reveal anterior wedging of three or more consecutive vertebral bodies that are located at the apex of the curve. Radiographic evidence of the in-plate erosion of the involved vertebrae often exists and schmoral nodules are common associated findings. For treatment, exercise and bracing are quite effective in treating mild structural kyphosis in the growing spine. However, when the deformity is severe and fixed, surgical correction and stabilization may be indicated. So here's just showing a normal spine with a normal vertebrae with the vertebrae being rectangle and shaped. And then this shows vertebrae with Sherman's disease. You're going to have more of a curvature or kyphosis with more wedge-shaped vertebrae. Next is Kleipel-Phil syndrome. This is a congenital malformation of the neck that results from a failure of segmentation in the developing cervical spine. Patients will exhibit a short broad neck, low hairline, and gross restriction of motion associated with other congenital malformations such as a springle deformity, rib deformity, scoliosis, central nervous system defects, cardiac anomalies, pulmonary anomalies, and renal anomalies. This can vary greatly in severity depending on the number of vertebrae that are fused. More severely affected individuals exhibit a short broad neck with appearance of webbing, a low hairline, and gross restriction of motion. Range of motion exercises or bracing may be tried to improve mobility or correct the deformity. Surgery except for cosmetic or the treatment of neurological dysfunction is rarely indicated. Next we're going to talk about congenital muscular torticollis. This is known as Rhineck. You're going to name the torticollis for the side of the head tilt and the head rotates away. So if the head is tilted to the left and rotated to the right it is going to be a left torticollis. This is produced by a fibrous and shortening of the sternocleidomastoid muscle thought to be caused by abnormal intrauterine positioning or due to birth trauma resulting in hematoma to the muscle belly of the sternocleidomastoid muscle. Usually it's recognized shortly after birth. There will be a swelling or a tumor that may be present in the sternocleidomastoid though this resolves after a few weeks with the fibrosis persisting. It results in a head tilt toward the affected side with rotation of the chin to the opposite side. These kids are going to commonly have hip dysplasia as well as plagiocephaly. This is just showing a picture with the head tilted toward the affected muscle with a contracted sternocleidomastoid muscle and then it's going to point away from the contracted muscle. Treatment consists of physical therapy. You can use orthoses like a top collar. They may also have plagiocephaly helmets. You can do Botox injections if there is a plateau in progression with therapy and also to prevent surgical correction. However will be used in the most severe cases. Next we're going to discuss spondylolisthesis. So this is a translation or forward displacement of one vertebral body over another. It's seen most commonly at the lumbosacral articulation. May develop as a result of insufficiency or fatigue fractures of the pars inter articularis. This is isthmic congenital dysplasia of the posterior spinal elements also known as dysplastic. Degenerative changes in the disc and facets and secondary to pathologic legions within the vertebrae and its elements. So an isthmic spondylolisthesis or spondylolosis is the most common type of spondylolisthesis. It's associated with low back pain that increases with strenuous activities and subsides with rest. They may have symptoms of nerve root irritation so radicular symptoms. On examination they're going to have loss of their lordotic curve. They could have tenderness of involved posterior elements, paravertebral muscle spasms, secondary tightness of their hamstrings. You may be able to note a step-off deformity at the spinous processes and range of motion is going to be limited in extension. Characterization and grading of the process are accomplished with radiographs. The oblique view may reveal a spondylolisthesis and the lateral view will show the degree of the spondylolisthesis. So you can see on the picture all the way to the right it's showing the different grades. Grades one through four of the slippage. Grade one is about 25 percent. Grade two is 25 to 50 percent. Grade three is 50 to 75 percent and grade four is 75 to 100 percent slippage. Treatment for mild to moderate cases will consist of exercising and bracing. You're going to work on strengthening the core muscles around the vertebrae and in the back. In cases where there's progressive slippage are going to require cervical fixation and those with neural involvement may also require a nerve root decompression. Next we're going to talk about herniated vertebral discs. This is not common in children and adolescents however it can be seen. It's almost always limited to the lower two segments of the lumbar spine. History of trauma is common. They're going to present with lower extremity radicular symptoms, pain, numbness, and weakness. Forward flexion, sitting, coughing, and straining aggravate the neurological symptoms. On exam they could have a straight straight leg test positive. They may have pain with flexion. They're going to have loss of the normal lumbar lordotic curve. They may have subtle abnormalities with reflex testing, motor, and sensory. Diagnosis, radiographs usually are normal so this diagnosis is verified by either CT or MRI. Treatment consists of non-surgical and surgical options. Non-surgical treatment consists of rest insets and therapy whereas surgical is reserved for more profound neurological deficits that persist. This includes a disc excision. That concludes this presentation. Listed here are the resources that were used. Thank you for your time.

pediatric spinal disorders

scoliosis

kyphosis

congenital muscular torticollis

spondylolisthesis

herniated vertebral discs