A Mild truncal asymmetry These mild asymmetries are variations of normal, do not require treatment, and cause no disability.

Normal Development

The axial system develops during the embryonic period.

Embryo

During the fourth week, mesenchymal cells from the sclerotome grow around the notochord to become the vertebral body and around the neural tube to form the vertebral arches [B]. Cells from adjacent sclerotomes join to form the precursor of the vertebral body, an intersegmental structure. Between these bodies, the notochord develops into the intervertebral disc. Cells surround the neural tube to become the vertebral arches.

During the sixth fetal week, chondrification centers appear at three sites on each side of the mesenchymal vertebrae. The centrum is formed by the coalition of the two most anterior centers. Chondrification is complete before the ossification centers appear [C]. The centrum, together with an ossification center of each arch, make a total of three primary ossification centers for each vertebra.

Childhood

During early childhood, the centers of each vertebral arch fuse and are joined to the vertebral body by a cartilaginous neurocentral junction. This junction allows growth to accommodate the enlarging spinal cord. Fusion of the neurocentral junction usually occurs between the third and sixth years. Anterior notching of the vertebrae is sometimes seen in the infant’s or child’s vertebrae and shows the site of somite fusion [C].

Secondary ossification centers

develop at the ends of the transverse and spinous processes and around the vertebral end plates at puberty. These fuse by age 25 years. Congenital defects are common in the axial system. Variations in the lumbar spine occur in about one-third of individuals. Spina bifida occulta is common. Hemivertebrae result from a failure of formation or segmentation. Such lesions are frequently associated with genitourinary abnormalities and less frequently with cardiac, anal, and limb defects; tracheoesophageal fistula; and conductive hearing defects if the cervical spine is involved.

Cord level

Initially, the neural and bony elements of corresponding somites lie opposite each other. Thus, the caudal end of the spinal cord fills the spinal canal, and the spinal nerves pass through the corresponding intervertebral foramina. By the 24th fetal week, the cord ends at S1; at birth, at L3; and in the adult, at L1 [A]. This differential growth rate results in the formation of the caudal equina: the accumulation of the nerves traversing the subarachnoid space to the intervertebral foramina. The end of the cord is attached to the periosteum opposite the first coccygeal vertebra by the filum terminale. The filum is the residual of the embryonic spinal cord.

Sagittal configuration

In the frontal projection, the spine is relatively straight throughout growth. In the lateral projection, the spine evolves from a single curve at birth to a triple curve pattern in the child [B]. Although this triple curve pattern is necessary to assume an upright posture, the obliquity imposes an added load on the lumbar spine. This load contributes to development of spondylolysis in the child, intervertebral disc herniation in the adolescent, and degenerative arthritis in the adult.

Evaluation

The spine is evaluated as part of a screening examination or to assess pain or deformity.

History and Physical Examination

Screening examination

Is there some underlying disorder? Marfan syndrome, neurofibromatosis, osteochondrodystrophies, or mucopolysaccharidoses are readily obvious in the older child but may not be so apparent in the infant.

History

Inquire about the onset, progression, disability, and duration. Family history is of great importance because scoliosis and hyperkyphosis are often familial. Back pain is also familial.

Posture

Note asymmetry of shoulder height, scapular prominence, flank crease, or asymmetry of the pelvis. Note any skin lesions, especially those in the midline. The presence of midline skin lesions, such as dimples, hemangioma or hair patches, cavus feet, or leg atrophy, are often associated with underlying spinal lesions. Café au lait spots are associated with neurofibromatosis, a cause of scoliosis.

Be aware that minor truncal asymmetries occur in about 10% of children. These are benign, cause no disability, and require no treatment. Avoid calling attention to such normal asymmetries because it only worries the patient and family.

Forward bending

Perform the forward bending test [A]. This is best done with the examiner seated in front or behind the child. Control the child’s forward bend by holding the hands together. Slowly guide the child’s forward bending while observing the symmetry of each level of the spine. Any significant scoliosis will be readily apparent.

For children unable to stand, examine the child sitting with forward bending. If unable to sit, examine the child prone and look for truncal asymmetry.

Assess asymmetry with a scoliometer [B], which measures inclination. Minor degrees of asymmetry are usually only a variation of normal, but require follow-up examination. If any abnormalities are found, a detailed physical and screening neurological examination is essential to avoid diagnostic errors. Hesitation, a list to one side, or restricted motion is abnormal. Lesions such as spinal cord tumors, spondylolisthesis, disc herniations, or discitis limit the mobility or symmetry on forward bending.

Side view

As viewed from the side, the back should curve evenly without any sharp angulation. A sharp angular segment of the spine is seen in Scheuermann kyphosis.

Neurological examination

should be part of the examination. In addition to the routine assessment, assess abdominal reflexes. Abdominal reflexes are assessed by gently stroking each quadrant of the abdominal wall [C]. Absence or marked asymmetry suggests a subtle neurological abnormality that may indicate the need for more intensive neurological investigation, such as MRI.

Imaging Studies

Radiographs and other imaging studies are indicated to measure the vertebral curves and to further assess specific problems identified by the physical examination [D].

Radiographs

Make PA and lateral spine films in the upright position on 36-inch film using shielding and techniques that avoid excessive radiation exposure. Order oblique lumbosacral views to assess the pars if spondylolysis is suspected and not seen on lateral view.

SPECT

Single-photon emission computed tomography (SPECT) imaging is useful to assess subtle pars reactions.

CT studies

are useful to detail bony deformities or lesions.

MR imaging

is used to study patients with neurological findings, those with unexplained progression of deformity, and certain types of deformity, as well as preoperatively for children with neurological impairment. These studies are helpful in evaluating tumors, congenital abnormalities such as Chiari malformation [C], various cysts, tethered cords, and filum terminale anomalies.

Multiple studies

Order only those imaging studies that are definitely necessary during the initial evaluation [D].

Normal variability

Measurements of deformity are made from standing radiographs [A]. Be aware of the normal range in the sagittal plane [B].

Sagittal plane

The normal range for dorsal kyphosis falls between about 20° and 45°. Kyphosis between 45° and 55° is marginal. Kyphosis below 20° is referred to as hypokyphosis, and above 55° as hyperkyphosis. Hyperkyphosis is sometimes referred to as a “round back” deformity. Normal levels for lumbar lordosis fall between 20° and 55°. Likewise, reduced lordosis is termed hypolordosis, and increased lordosis is termed hyperlordosis. Hypolordosis is called a “flat back” and hyperlordosis either a “lordotic deformity” or a “swayback.”

Frontal plane

Mild curves that cause truncal asymmetry are usually normal variants. These variations are <10° by Cobb and <5° by scoliometer measure. These asymmetries have not been shown to cause any disability in childhood or adult life. Bone scans are usual in assessing back pain when radiographs are negative or equivocal.

Congenital Deformities

Diastematomyelia

This is a congenital defect with a central cartilaginous–bony projection that divides the spinal cord [A].

Diagnosis

Cutaneous lesions occur in most patients with a hairy patch, dimple, hemangioma, subcutaneous mass, or teratoma at or near the level of the diastematomyelia. Other deformities are common. Nearly all have some associated anomaly, such as spinal dysraphism, asymmetry of the lower extremities, clubfoot, or a cavus foot. Two-thirds have congenital scoliosis. Two-thirds are located in the lumbar spine. Half have neurological abnormalities.

Management

Resect the spur in a patient with progressive neurological findings. Follow the others and consider resection should neurological findings develop or if correction of spinal deformity is planned.

Sacral Agenesis

Caudal regression or sacral agenesis includes a spectrum of abnormalities [B] with hypoplasia or aplasia [C] of the sacrum, which is most common in offspring of diabetic mothers.

Clinical features

include knee-flexion contractures with popliteal webbing, dislocations and flexion contractures of the hips, scoliosis, equinovarus deformities of the foot, and instability at the spinal–pelvic junction. These deformities vary in severity with the level of the agenesis and the resulting loss of motor power. Neurological features may be most predictive of progression, and MR imaging is helpful in assessment.

Management

is often difficult and depends upon the deformity, motor, and sensory status. Knee flexion deformities are difficult to correct, and recurrence is common. The combination of limited operative procedures and orthotic or mobility aids are tailored to the child. Spine–pelvic instability and hip dislocations are often better tolerated than the stiffness caused by surgical stabilization or reduction.

Exstrophy Bladder

A failure of anterior closure of the pelvis results in pelvic diastasis and an open bladder [D]. In the more severe form, cloacal exstrophy, an omphalocele containing intestinal contents, is also present.

Clinical features

include pelvis diastasis, acetabular retroversion, and lateral rotation of limbs with out-toeing gait. This out-toeing tends to improve with age.

Management

Orthopedic disabilities are insufficient to require correction. A pelvic osteotomy may be required during bladder reconstruction to facilitate closure. Perform bilateral supra-acetabular osteotomies and stabilize with a spica cast following urological repair.

Back Pain

During the past several decades, back pain in children has become increasingly common [A]. This is especially true in adolescents and late teens. This increase is associated with increasing stress, complexities in family relationships [B], diminished physical activity, and increasing time with television and electronics. Because of the prevalence and social/cultural factors, the role of the primary care provider has increased significantly.

Prevalence

Back pain has becomes increasingly common especially among girls in late teens [A].

Evaluation

Be aware of the causes of back pain [C]. Worrisome features include onset before age 4 years, symptoms persisting beyond 4 weeks, interference with function, systemic features, increasing discomfort, night pain, neurological findings, and recent onset of scoliosis.

The location is significant [D]. Thoracic back pain is more common in girls, more common in late teens, and much less likely to be serious and require treatment.

Perform a careful physical examination. On bending, look for symmetry, mobility, and evenness of the curvature. Note the location of any tenderness. Perform a neurological examination and check for tight hamstrings.

Generally, image by radiographs – anteroposterior and lateral studies – are sufficient. Be aware that oblique views and CT scans expose the child to significant amounts of radiation. Avoid ordering MRI and other expensive studies. The spine doctor will prefer to order exactly what is needed.

When to refer

Upper thoracic pain

Can often be managed in primary care.

Low back pain

Especially if associated with localized tenderness, limited spine mobility, limited straight leg raising, uneven curvature of the back when bending, or asymmetry.

Management

Adolescent benign back pain

Some suggest limiting backpacks to less than 20% of body weight (not evidence based). Encourage activity, a healthy lifestyle, and weight control. Provide reassurance. Consider this as a common backache, requiring no treatment, and best ignored.

Prognosis

If back pain is present in adolescence together with a positive family history of back pain, nearly 90% of these adolescents will have back pain in adult life. Psychosocial problems are more significant than structural abnormalities in determining the likelihood that back pain will become chronic [C].

Tumors

Tumors may be metastatic or primary. Primary tumors may arise from the cord or bone [A].

Metastatic Tumors

These tumors are most common in the thoracic, then lumbar, and least common in the cervical spine [B]. Manage with chemotherapy and radiation. Mortality is high. Those who survive are likely to have deformity. Early stabilization may prevent progression of the deformity.

Primary Tumors

Primary tumors may occur in the vertebrae or cord. Most vertebral tumors are benign, and most cord tumors are malignant. Either type may cause spinal cord compression [C].

Spinal cord tumors

cause diagnostic difficulties. They may present to the orthopedist with torticollis, scoliosis, gait disturbances, foot deformities, or back pain. Often forward bending is limited and asymmetrical. Perform a careful neurological examination. Study with plain radiographs first. Look for changes in intrapedicular distance. MRI studies are usually diagnostic.

Vertebral tumors

are more common. Most are benign. Most present with pain. Duration of symptoms from benign tumors is usually longer than those from malignant tumors. Most may be diagnosed by conventional radiographs.

Osteoid osteoma and osteoblastoma

cause classic night pain, usually secondary scoliosis, limited spinal mobility [D], often tenderness, and sometimes classic radiographic features. Bone scans are often diagnostic. Excision is often necessary. Exactly localize with preoperative imaging. Percutaneous ablation is an option [E].

Eosinophilic granuloma

causes pain, tenderness, limited mobility, and usually a focal lesion. The classic vertebrae plana [F] is often absent. For solitary, uncomplicated lesions, observational management is appropriate. If lesions are multiple or if neurological involvement is present, operative resection may be necessary.

Aneurysmal bone cysts

cause pain, rarely cord or root compression, sometimes deformity, and limited mobility. Radiographs are often diagnostic with expansion and ballooning of the cortex [G]. Management is often difficult. Manage with preoperative selective arterial embolization, intralesional excision curettage, bone grafting, and fusion of the affected area if instability is present.

Scheuermann Disease

Scheuermann disease is a familial disorder of the thoracic spine producing vertebral wedging and kyphosis greater than about 45° [A] and often causing back pain.

Clinical Features

A history of heavy physical loading from athletics or work is common. Often the deformity is familial [B]. Patients often complain of deformity, fatigue, and sometimes pain. The normal even contour of the spine is lost with an abrupt kyphotic segment at or above the thoracolumbar level. Tenderness over the apex may be present. Radiographs show anterior body wedging. Mild scoliosis is common. The strict definition requires a wedging of at least 5° involving three vertebrae [C].

Management

Treat the pain by NSAIDs, rest, and stress reduction. Sometimes a thoracolumbar sacral orthosis (TLSO) will be helpful in controlling the pain. Management of the deformity is discussed on page 253.

Schmorl Nodes

These nodes are vertical herniations of the intervertebral disc through the vertebral endplate, causing narrowing of the disc space [D]. Sometimes the condition is referred to as lumbar Scheuermann disease. This herniation is most common in adolescents, is often associated with trauma, and may be the cause of back pain. The lesions may be seen on plain radiographs, although MR imaging is most sensitive and may be indicated when the diagnosis is uncertain. Manage by rest, NSAIDs, and sometimes a TLSO.

Disc Herniation

Disc herniations occur rarely in adolescents. Predisposing features include positive family history, recent trauma, facet asymmetry, spinal stenosis, transitional vertebrae, and spondylolisthesis.

Clinical Features

Herniations usually occur at the L4–L5 or L5–S1 levels, often producing radicular pain and secondary spinal deformity. The patient may be seen because of scoliosis or a list. Straight leg raising is limited, and neurological changes are variable. Radiographs are usually normal. Occult spina bifida is more common in these patients. MRI studies or myelography shows the lesion [E]. Disability is increased if the herniation is associated with spinal stenosis. Be aware that fracture of the lumbar vertebral ring apophysis may be confused with disc herniations.

Management

Manage first with NSAIDs, rest, limited activities, and a TLSO. Persisting or increasing disability are indications for MRI and operative disc excision. Endoscopic or open discectomies are successful in 90% of cases.

Discitis

Discitis is an inflammation (probably an infection) that involves the lower thoracic or upper lumbar disc spaces in infants and children. Unlike other musculoskeletal infections, discitis usually resolves spontaneously.

Clinical Features

The clinical features of discitis are age related. Discitis in the infant is characterized by fever, irritability, and an unwillingness to walk. The child may show constitutional illness with nausea and vomiting. The adolescent may complain of back pain. Because the symptoms are vague and poorly localized, the diagnosis is often delayed. The findings of fever and malaise, a stiff back, unwillingness to walk, and an elevated ESR and CRP are suggestive of discitis.

Imaging

Early in the disease, a bone scan may show increased uptake over several vertebral levels [A]. After 2–3 weeks, narrowing of the disc space is seen on a lateral radiograph of the spine. MRI often shows worrisome features and may lead to overtreatment [B].

Aspiration or biopsy

Disc space aspiration is not necessary unless the disease is atypical.

Management

Manage based on the stage and severity of the disease. If the child is systemically ill, antistaphylococcal antibiotic treatment is appropriate. If the child is acutely ill, an intravenous route is appropriate. Otherwise, oral medication is adequate. Discitis is more severe in the older child [C]. Continue antibiotics until the ESR returns to normal. For comfort, consider immobilization in a “panty spica” or brace [D] for a period of several weeks.

Prognosis

Long-term studies show a variety of abnormalities that include residual narrowing [A, right], block vertebrae, and limited extension, but the likelihood of back pain is not increased.

Spondylolysis and Spondylolisthesis

Bilateral or unilateral defects of the pars interarticularis cause spondylolysis [A]. This defect may allow displacement of the vertebrae, which is called spondylolisthesis. These lesions are the most common cause of structural back pain in children and adolescents.

Pathogenesis

In children, these conditions are usually due to a stress fracture through a congenitally dysplastic pars interarticularis. This inherent weakness occurs more commonly in certain races (such as Inuit peoples), families, or individuals. The defects often are associated with spina bifida occulta. Spondylolisthesis occurs in about 4% of 4-year-old children and increases to about 6% by maturity. Spondylolisthesis occurs in about a third of those with pars defects, especially in those with mechanical instability. These lesions occur more commonly in children with abnormal bone or connective tissue, as occurs in conditions such as Marfan syndrome and osteopetrosis. Lesions are common in children who participate in certain sports that cause hyperextension of the lumbar spine with rotation, such as gymnastics, wrestling, diving, and weightlifting [B]. Progression after adolescence is unusual.

Clinical Features

History and physical examination

The child usually complains of back pain. Tenderness may be present at the L5-S1 level. If the displacement is severe, a prominence is palpable over the defect. Straight leg raising and forward bending may be limited. The neurological examination is usually normal. If the condition is acute, secondary scoliosis may be present.

Imaging

First, order a standing lateral radiograph of the lumbosacral spine. A forward displacement of the body of L5 or L4 establishes the diagnosis. If no displacement is present, order oblique radiographs of the lower lumbar spine to assess the status of the pars. Spina bifida occulta is common in children with the defect. A bone scan may show reaction [C] before radiographs show a defect and may be used to determine the activity and healing potential of the lesion. Even more sensitive is SPECT imaging in demonstrating the stress reaction of spondylolysis.

Classification

Wiltse classifies spondylolisthesis into two types:

Dysplastic

is a congenital facet deficiency allowing slippage.

Isthmic

allows slippage due to a defect in the pars interarticularis. These lesions may be due to a fatigue fracture, a stress fracture, or elongation without fracture.

Grade

the degree of slip in severity and activity (duration).

Severity

Grade on the basis of slip angle [A, next page] and displacement [C, next page]. Slip angle changes usually occur with slips greater than 50%.

Activity

Grade on the duration or activity. Recent fractures are active and show increased uptake on bone scan. Cold lesions are chronic, inactive, and less likely to heal.

Progression

Pain is most pronounced at the time of onset or fracture. Most isthmic lesions become stable and painless with time. Pain is aggravated by activity, especially competitive sports. Lesions often are symptomatic in adolescence but become painless in adult life when activity levels are reduced. The incidence of back pain is comparable to normal population levels.

Management

Management is based on the patient’s age, degree of deformity, type of lesion, activity, and physical activity level.

Spondylolysis

management depends upon the activity of the lesion.

Acute lesions

from an acute injury or recent overuse experience are managed by reduction of activity and usually an under-arm brace [B]. Often these lesions will heal.

Established lesions

Manage symptoms with NSAIDS and activity modification. Operative stabilization is seldom necessary.

Spondylolisthesis

is managed based on the severity of the slip, considering the displacement and slip angle. If fusion is required, it is often performed without reduction.

Grade 1–2 slips

Manage with NSAIDS, activity modification, and TLSO as necessary to control symptoms. Follow with standing lateral radiographs.

Grade 3 slips

Most require operative stabilization in children. Fuse L4–S1 level with posterolateral autogenous grafting.

Grade 4 slips

These slips may require fusion of L4-S1, as the displacement may be significant, making identification of the transverse process of L5 difficult. If slip angle is severe, reduction is sometimes performed.

Grade 5 (spondyloptosis)

management is controversial. In situ fusion provides pain relief and safety, but the deformity remains. Reduction incurs greater risk but improves appearance and posture.

Special situations

require tailoring of management.

L4 spondylolisthesis

is less common, more mechanical in etiology, often causes more symptoms, and is more likely to require operative stabilization.

Spondylolysis

with persisting symptoms may be managed by repair of the pars defect with grafting and fixation.

Scoliosis

Scoliosis is often defined as simply a frontal plane deformity of the spine >10°. The deformity is much more complex, however, and includes significant transverse and sagittal plane components. The causes of scoliosis are numerous [A]. Mild truncal asymmetry occurs in as much as 10% of the population and may be considered as a variation of normal. Curves greater than 10° are abnormal, and in the growing child may progress to cause a significant problem. Scoliosis is the most common back deformity.

Secondary or Functional Scoliosis

This type of scoliosis can also be described as “functional” because it is secondary to some other problem [B]. The scoliosis usually resolves when the underlying problem is corrected. The scoliosis is usually flexible and nonstructural. There are no bony changes, and the rotational elements are minimal. The common causes of functional scoliosis are leg length inequality and muscle spasm.

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