Chapter 216. Disorders of the Neck and Spine

Torticollis refers to lateral tilt and rotation of the head and neck. The most common form of torticollis is known as congenital muscular torticollis and is due to “over pull” of the sternocleidomastoid muscle to the side of head tilt. So, for example, a right-sided sternocleidomastoid muscle contracture would result in head tilt to the right and rotation of the chin to the left (Fig. 216-1). This deformity is seen at birth and is often associated with a very tight sternocleidomastoid muscle (SCM) that often has a small mass or swelling in its midsubstance which is palpable but not painful. The incidence of congenital muscular torticollis is 3 to 5 per 1000 births. The etiology is not completely understood but is associated with breech presentation and difficult forceps delivery and occurs more commonly in first-born children. It is important to remember that this is a painless torticollis. Other associated orthopedic conditions are associated with torticollis including hip dysplasia and metatarsus adductus. These conditions should be evaluated as part of the normal orthopedic examination with specific focus placed on the examination of the hips and feet, as well as the spine in patients with muscular torticollis.1

The clinical presentation typically demonstrates a predisposition to tilting the head to one side with rotation to the opposite side. A small mass in the midportion of the sternocleidomastoid is palpable on the affected sternocleidomastoid muscle and has the feel and size of an olive. The face on the ipsilateral side of the SCM lesion demonstrates some flattening with loss of roundness of the back of the head (plagiocephaly) on the opposite side.

The differential diagnosis of congenital muscular torticollis includes congenital cervical vertebral anomalies, inflammatory atlantoaxial rotatory displacement, central nervous system (CNS) tumors of the posterior fossa or cervical spinal cord, or visual abnormalities. When the sternocleidomastoid is contracted with the palpable olive with a consistent head tilt to the ipsilateral side with rotation to the opposite side, the diagnosis is nearly confirmed. Treatment consists of stretching exercises to rotate the infant’s chin to the ipsilateral shoulder, while tilting the head toward the contralateral shoulder. This should be performed several times during the day and generally results in outstanding correction of this deformity over a 6 to 12 month period of time. Other modalities such as positioning the child in their crib so as to look at objects opposite to his or her restricted motion are appropriate. Early nonoperative treatment is usually successful with restoration of full motion.

The plagiocephaly seen with congenital muscular torticollis is due in large part to the abnormal head tilt and rotation, as well as the supine position that has been recommended by the American Academy of Pediatrics of 1992. It is exceptionally uncommon for this plagiocephaly to result in facial asymmetry requiring any type of treatment. In general, as the torticollis improves, plagiocephaly improves because asymmetric pressure is alleviated with treatment. There is some evidence that use of an orthotic helmet improves this plagiocephaly; however, well-controlled studies seem to be lacking. Surgical treatment for congenital muscular torticollis is uncommon, if aggressive physical therapy is begun early. Surgical treatment requires lengthening of both the proximal and distal ends of the sternocleidomastoid to improve the position of the head and generally results in improved head positioning but does not generally result in completely normal range of motion treatment.

Klippel-Feil syndrome presents as a spectrum of congenital fusions or failure of formation of the cervical vertebrae. Klippel-Feil syndrome is defined as the triad of a short, webbed neck, combined with low posterior hairline and fusions in the cervical spine. It is important to recognize Klippel-Feil syndrome because neck mobility is limited and may predispose the patient to a cervical spine injury with some traumatic event. In addition, Klippel-Feil syndrome can be associated with an elevated scapula (Sprengel’s deformity), which should be recognized at the time of the evaluation. Finally, scoliosis below the cervical spine either due to congenital abnormalities in the thoracic or lumbar region or associated compensatory curves below the cervical congenital scoliosis can be seen. Sprengel’s deformity is seen in up to 50% and scoliosis is seen in 30% of patients with Klippel-Feil syndrome. In addition, because the intrauterine development of the spine occurs at a similar time, the genitourinary system should be evaluated with renal ultrasound to ensure normal development.2

The clinical examination will demonstrate a low hairline with a short, webbed neck and restricted range of motion. There is a wide spectrum of motion because only a mild single-level fusion of the cervical spine is defined as Klippel-Feil syndrome, and almost-complete fusion is given the same diagnosis. Radiographic features include cervical spine fusions in either single levels or multiple levels, or there may be two blocks of vertebrae separated by a single motion segment. A spot lateral radiograph is ideal to identify the deformities and fusions (Fig. 216-2); however, anteroposterior (AP) and open-mouth views are also necessary for a complete initial evaluation of the cervical spine. Visualization of the entire thoracic and lumbar spine is also necessary to fully evaluate any scoliosis in these regions of the spine. Referral to the orthopedist is necessary for full evaluation of these deformities and would include a neurologic examination to assess for upper motor neuron signs or other neurologic deficits. Lateral radiographs of the cervical spine with the head positioned in flexion and extension are important to look for cervical spine instability. Further evaluation with magnetic resonance imaging (MRI) is important, especially for those patients who demonstrate neurologic abnormalities or who have cervical spine instability.

Patients with Klippel-Feil syndrome usually only require education of the patient and family to refrain from high-energy activities that may lead to cervical neck injuries. Surgical treatment is necessary only when there is increasing or painful deformity or neurologic deficits. Surgical decompression of the cervical spinal cord is necessary when neurologic deficits are present, and fusion to stabilize the spine is important if progression of the spine deformity occurs or there is instability.

The spine has normal sagittal plane contours to include lordosis (swayback to the cervical spine). When kyphosis occurs, it can be due to many causes including congenital abnormalities that are most often associated with syndromes such as Larsen’s syndrome, diastrophic dysplasia, camptomelic dysplasia, and neurofibromatosis. These cervical kyphoses are important to recognize because they can result in constriction of the spinal cord and neurologic deficits. Any young patient should be evaluated for a syndromic condition by a dysmorphologist or a geneticist. Treatment is necessary for progressive deformity or when neurologic deficits occur. Surgical treatment is the only definitive treatment and requires either posterior fusion or anterior and posterior fusion combined with decompression when neurologic problems occur. Iatrogenic kyphosis is most often due to a previous removal of the posterior elements to gain access to the neural elements for tumors, infection, or syringomyelia. The loss of the posterior elements leads to kyphosis which can result in neurologic deficits.

A lateral curvature of the spine with rotational deformity in the axial spine is true scoliosis. Those with scoliosis can be divided into those who have an underlying neurologic condition and subdivided into upper motor neuron (cerebral palsy, Charcot-Marie-Tooth disease) and lower motor neuron (spinal muscular atrophy, myelomeningocele). Myopathic conditions include Duchenne’s muscular dystrophy and arthrogryposis. Connective tissue conditions include Marfan’s syndrome and osteogenesis imperfecta. One of the most common miscellaneous categories includes neurofibromatosis. Congenital scoliosis refers to a failure of segmentation (separation) or formation of the vertebra which distorts the spine by the absence of normal rectangular vertebrae. The most common type of scoliosis seen is idiopathic in which an identifiable cause cannot be found.

Idiopathic Scoliosis

This is the most common type of scoliosis, and it can be divided by age group into infantile (0 to 3 years), juvenile (3 to 10 years), and adolescent (> 10 years).3 The vast majority of patients with idiopathic scoliosis present secondary to an observed abnormal appearance in their spine which is visualized as a shifting of the body to one side, waistline asymmetry, shoulder height differences, or a prominence seen in the back. Scoliosis can be defined as a lateral curvature of the spine > 10°, with rotational deformity as the hallmark of this spinal curvature.

The incidence of scoliosis in the adolescent period is approximately 2% with only 0.6% of patients requiring treatment. Adolescent idiopathic scoliosis (AIS) is more commonly seen in girls, especially when the curves are larger. Although the etiology of idiopathic scoliosis is not known, we know that AIS curves progress in two main ways: first, with continued spinal growth curves tend to progress, and second, if curves are > 45° to 50° at the time of skeletal maturity, curve progression in adulthood is likely.

Infantile and juvenile scoliosis differ from the more common adolescent scoliosis in that curve progression is more likely because patients are younger and underlying neural axis abnormalities (ie, syringomyelia, tethered cord, diastatometamyelia) are seen in 20% of patients. An MRI of the entire spine is necessary to identify these abnormalities. The position and angle of the ribs relative to the spine at the apex of the deformity are used to predict curve progression.

Clinical Features

Patients with idiopathic scoliosis do not present with symptoms of pain. The deformity is noticed by family members, as well as nurses when school screening is performed, or by the pediatrician during annual visits. The physical examination is best performed with the patient standing in a relaxed position with feet together and arms at his or her sides. The patient is visualized from the back to look for shoulder height asymmetry, waistline asymmetry, or prominences in the back secondary to the rotational deformity. The Adam’s forward bend test is then performed by having the patient bend forward at the waist with the arms dangling in front in a very relaxed position. The rotational deformity of the spine is easily visualized or palpated (Figs. 216-3, 216-4, and 216-5). Pain with palpation may indicate a diagnosis other than idiopathic scoliosis, and further imaging will be necessary. A careful neurologic examination is always performed in a patient with scoliosis to include a good motor examination, sensory examination, and most importantly, reflex examination. The most reliable reflex examination is the presence of abdominal reflexes in which the abdomen is stroked from medial to lateral. The umbilicus should move toward the stroked side. Symmetric (right and left) presence or absence of these reflexes is considered normal, and asymmetry or absence suggests neurologic abnormality.

During the Adams forward bend test, a scoliometer can be utilized to objectively measure the rotational deformity. In general, patients with curves > 7° on the scoliometer reading should be referred to the orthopedist. This usually represents a 20° coronal radiographic curve measurement.

Radiographic imaging can be performed but is often done at the orthopedist’s office. This includes a standing posteroanterior (PA) view, as well as a lateral view of the spine to include the cervical, thoracic, lumbar, and sacrum. On the PA view, the curves are measured using the Cobb method, which is performed by measuring the largest curve, and angular measurements are then determined (Fig. 216-6). Other important parameters on the radiographs include assessment of the Risser sign, the status of ossification of the iliac wing apophysis which correlates with the amount of remaining spinal growth. A Risser stage 0 or 1 indicates significant remaining spinal growth, and a Risser stage 4 or 5 indicates little remaining growth. Rapid spinal growth is occurring when the growth plate of the acetabulum (triradiate cartilage) is open and when girls are premenarchal.

Treatment

Treatment for idiopathic scoliosis is 3-fold. First, observation with serial radiographs every 4 to 6 months is performed in adolescents for curves < 25° with continued growth remaining. Bracing is utilized for curves between 25° and 45° in those children who are growing rapidly. The goal of brace treatment is to prevent further curve progression to avoid the need for surgery. The effectiveness of bracing is due to its ability to correct the curve while the patient is wearing the brace, so that further progression does not occur. It is important to realize that the braces utilized today go under the clothes, stay below the arms, and do not need to be seen by others. Two main types of braces are used depending on the curve type and curve magnitude. The TLSO (thoracolumbar-sacral orthosis) or Boston brace can be utilized for all curve types with curve magnitudes between 25° and 45° and is worn both during the day and at night. The bending brace or Charleston brace is good for thoracolumbar or lumbar curves between 25° and 35° and is only worn at night. The final option is surgical treatment, which is utilized for curves > 45° to 50° because curve progression into adulthood is likely. The goal of surgical treatment is to prevent further curve progression while gaining significant correction of the spinal deformity. The most common curve type is a right thoracic curve. Preoperatively the patient needs a thorough evaluation. Indications for MRI include a neurologic abnormality (most commonly, is asymmetrical abdominal reflexes), a lower extremity asymmetry or a cavus foot deformity, severe painful scoliosis, or an atypical curve pattern such as a left thoracic curve. An MRI analyzes the neural axis for abnormalities such as a syringomyelia, diastematomyelia, or tethered cord that will then be managed neurosurgically.

Preoperative radiographs include a standing AP and lateral x-ray of the spine, as well as flexibility x-rays to identify those curves that are truly structural and not just “compensatory.” A compensatory curve is opposite in direction to the main structural curve, attempting to keep the patient in an overall balanced situation. For example, a right thoracic curve can have a left lumbar compensatory curve to prevent the patient from having a severe right trunk shift. Only the curves deemed structural are included in the operative procedure.

Surgical treatment can be performed from the posterior approach, the anterior approach, or with a combined anterior and posterior approach. The indications for a combined approach (anterior/posterior) are when a large curve magnitude is present (> 80°) or when the patient is very young (< 11 years old) with significant remaining spinal growth. The anterior approach is used to remove disc material to improve the flexibility of the spine for large curves and achieves anterior fusion, preventing further anterior spinal growth and return of the curve in the very young patient. The addition of an anterior procedure is relatively uncommon today given the advances in posterior instrumentation techniques. The posterior approach utilizes anchor points to the spine which then become attached to two rods. These implants are used to partially correct the spine and then hold the spine until the vertebrae fuse together in the corrected position (Figs. 216-7 and 216-8). Fusion of the spine then results in decreased motion of the spine, which is generally not perceived by the patient; however, the long-term health of the spine may be somewhat compromised, because the remaining motion segments function and move to a greater degree. In general, surgery for AIS achieves between 50% and 70% curve correction depending on many factors including the flexibility of the curve, whether compensatory curves are present, and the status of the spinal cord monitoring at the time of surgery.

Overall, surgical treatment for idiopathic scoliosis has a high success rate. Potential complications include infection, both acute as well as delayed infections, pseudarthrosis in which one of the motion segments of the spine fails to fuse, and neurologic deficits that are extremely uncommon. State-of-the-art intraoperative spinal cord monitoring is utilized and includes monitoring of the sensory tracts as well as the motor tracts of the spinal cord. The overall complication rate for spine surgery in a patient with AIS is between 4% and 5%, and the incidence of neurologic deficit is less than 1%.

Congenital Scoliosis

Scoliosis due to an abnormal formation or separation of the vertebrae during development is termed congenital scoliosis. The spine develops during the eighth week of gestation, at which time the renal and cardiac systems also form, so it is important for these systems to be evaluated when congenital scoliosis is seen. The most common congenital abnormalities are failure of formation resulting in a hemivertebrae (a triangular-shaped vertebrae) or failure of segmentation, in which a bar of bone connects two or more vertebrae. These spinal deformities can lead to severe scoliosis and are also associated with congenital rib abnormalities and fusions that may result in constriction of the chest leading to pulmonary issues.4

Patients with congenital scoliosis present in many ways, and treatment may need to be aggressive very early or may only require observation, depending on the overall curve magnitude and the risk for progression. A congenital hemivertebrae that has growth plates on both sides of the vertebrae combined with concave tethering (secondary to a congenital bar) has a high risk for progression, but a single hemivertebra without growth on both sides is less likely to progress (Fig. 216-8). An evaluation of the spinal cord with MRI is necessary because these patients have a higher likelihood of having neural axis abnormalities. Treatment for congenital scoliosis can be very complex, and referral to an orthopedic surgeon at the time of diagnosis is important. Various options for treatment include excision of the hemivertebrae with fusion above and below that segment, use of growing rods requiring surgical lengthening of the rods every 6 months, or placement of a vertical expandable prosthetic titanium rib (VEPTR) when chest wall and rib deformities are the predominant issues.

Neuromuscular Scoliosis

Any patient with a neuromuscular condition, such as cerebral palsy, myelomeningocele, or Duchenne muscular dystrophy, is more likely to develop scoliosis secondary muscle imbalance or weakness. These scoliosis curves can occur at any time but are most common during the adolescent period. They are usually long, sweeping, C-shaped curves that generally span the entire spine (Fig. 216-9). For those patients who are nonambulators, bracing can be occasionally utilized; however, the most effective nonoperative treatment is wheelchair modifications to maintain the patient’s posture and prevent progression of scoliosis. Surgical treatment is common because curve progression is rapid and nonoperative methods to control the curve are challenging and often ineffective. Surgical treatment usually requires fusion from the proximal thoracic spine to include the pelvis to ensure adequate correction and maintenance of that correction over time.5

Normal thoracic kyphosis measured between T5 and T12 is between 20° and 45°. Hyperkyphosis refers to excessive roundback deformity. This deformity may be either postural, in which the spine is flexible and does not require treatment, or more severe and rigid, termed Scheuermann kyphosis. A distinction between the two is based on both the clinical and the radiographic examinations. A patient who has excessive roundback that self-corrects with hyperextension while standing, has a condition that is a variation of normal and does not need treatment. A patient, however, who has stiffness in the spine and a rigid kyphosis, requires further evaluation with radiographs. In Scheuermann kyphosis, a lateral radiograph will demonstrate a curve greater than 60° which has at least three consecutive vertebrae with: 5° of anterior wedging of the vertebral bodies. This is associated with decreased disc spaces, irregular endplates, and Schmorl nodes (slight disruptions or cavitations in the endplates of the vertebrae).

Patients with Scheuermann kyphosis often complain of pain over the apex of the curve or pain in the lumbar spine secondary to the compensatory lumbar hyperlordosis. Neurologic deficits from Scheuermann kyphosis are rare. However, this condition can be associated with herniated thoracic discs which may produce sensory loss in a thoracic nerve root distribution, or with congenital spinal stenosis, which is considered when the patient describes painful symptoms with prolonged walking.

Nonoperative treatment of Scheuermann kyphosis in a growing child includes either brace treatment or casting, both of which are rarely used today. Progression of Scheuermann kyphosis is not well understood. However, like idiopathic scoliosis, curve progression is seen with rapid spinal growth.

Surgical treatment for Scheuermann kyphosis is generally reserved for those patients who have demonstrated progression of the kyphosis despite skeletal maturity, or prolonged pain despite hyperextension physical therapy exercises, hamstring strengthening, and core strengthening. Generally symptoms do not begin until curves are > 70°, and surgical treatment is rarely necessary with smaller curves. Surgical treatment is similar to scoliosis treatment in that implants are utilized to correct the kyphosis while fusion occurs. In general, patients are very satisfied with surgical procedures, because the cosmetic appearance is significantly improved following this treatment. Similar potential complications are seen as in the spine surgery for scoliosis; however, a slightly greater neurologic risk is seen.6

Back pain in children and adolescents is common and can be divided into many causes. Often, however, identifying the cause of the pain is difficult. When an etiology is not identified, the back pain is termed “mechanical low back pain” and most probably results from muscle spasm as the paraspinal muscles fatigue during or following activities. It most commonly occurs at the end of a very active day.7 Important features are that the pain is localized to the low back without radiation into the lower extremities, is better with rest, and is not associated with bowel or bladder symptoms. Night pain that awakens the patient from sleep or constitutional symptoms including fevers, night sweats, or loss of weight, are the worrisome features that require an extensive workup.

Referral to the orthopedic surgeon is necessary with back pain that is not relieved by a good core strengthening and hamstring stretching program that is performed daily, or if worrisome symptoms are identified.

Evaluation generally begins with plain radiographs to include the entire spine when the back pain is generalized. Specific radiographs focused on the local area of pain are often performed. These often include a lateral x-ray of the lumbosacral spine to look for spondylolysis (microfracture of the spine) or spondylolisthesis (anterior slippage of one vertebra on the other) (Figs. 216-10 and 216-11). An oblique radiograph is very useful to identify the spondylolysis that is seen as a collar around the neck of the “Scotty Dog” and is most common at the L5 level followed by the L4 level. Treatment generally begins with immobilization in a brace with core strengthening and hamstring stretching exercises. Surgical treatment for this condition is uncommon and includes repair of the defect if it is L4 and fusion between L5 and S1 if the defect is at L5.8

Slippage of L5 on S1 is known as spondylolisthesis and occurs in approximately 3% to 6% of adolescents. It is more common with hyperextension sports, including gymnastics, weight lifting, and baseball pitching. Some patients have significant back pain, especially with hyperextension, and also have severe hamstring tightness. Neurologic symptoms can develop with very-high-grade spondylolisthesis (> 50% slippage of L5 on S1). The patients with more severe slips often present with extreme hamstring tightness and pain radiating to the lower extremities, especially with extension of the hips and knees.

A lateral radiograph demonstrates the spondylolisthesis that can be further characterized based on the etiology (dysplastic, elongation of the pars; lytic, microfracture of the posterior elements) and the amount of translation of the vertebrae on each other (low grade, less than 50% translated; high grade, greater than 50% translated).

Treatment of spondylolisthesis is with physical therapy for core-strengthening exercises, and hamstring stretches for low-grade slips. Surgical treatment is generally designated for those patients who have high-grade spondylolisthesis. The goal of surgical treatment is to obtain fusion to prevent further progression. The traditional method has been an in situ (in place) fusion between the L4 and S1 vertebrae, which is required to achieve fusion. The L4 is incorporated to allow for a more stable and more reproducible fusion, because L5 has slipped so far forward. If severe kyphosis at the L5 to S1 level occurs, then other treatments to include reducing the kyphosis as well as fusing with instrumentation may be more appropriate. This surgical treatment does risk neurologic deficits at the L5 nerve root.9

Herniated discs, or herniated nucleus pulposus (HNP), are extremely uncommon in children and adolescents. The HNP may impinge on neural structures, specifically nerve roots, and cause pain that radiates into the lower extremities into the foot. The L4-5 and L5-S1 levels are most commonly herniated. Physical examination demonstrates pain with straight leg raising to 80° and other nerve root tension signs. An MRI confirms a herniated nucleus pulposus. Treatment for children and adolescents is pain medication to relieve or improve symptoms to allow the patient to perform core-strengthening exercises. Generally, conservative management for HNP is successful but may be supplemented with epidural corticosteroids to reduce inflammation during this healing phase. Occasionally bed rest is warranted to achieve some pain relief. Surgical excision of the HNP is occasionally required with failure of conservative treatment.10

Certain benign tumors occur in the anterior vertebral body (eosinophilic granuloma and hemangioma), and others occur more commonly in the posterior elements (osteoid osteoma, osteoblastoma, and aneurysmal bone cyst).

The most common anterior benign bone tumor is eosinophilic granuloma that results in vertebral plana in which the vertebral body (normally rectangular) becomes extremely flat. This is a self-resolving condition; however, it is important to distinguish it from the malignant condition of Ewing sarcoma. The distinction is made based on more symptoms and a soft tissue mass on MRI that are associated with Ewing sarcoma and not vertebra plana.

Osteoid osteoma always occurs in the posterior elements and is associated with pain that typically occurs at night that is relieved with aspirin or nonsteroidal anti-inflammatory medications. A computerized tomography (CT) scan demonstrates the characteristic nidus (a central bony spicule) surrounded by bony reaction. Osteoid osteoma will resolve over time; however, symptoms can persist for many years, and if these are not tolerated by the patient, then surgical excision can be performed.

Aneurysmal bone cyst is an outpouching of the posterior elements which may cause neurologic problems. This can be very challenging to treat but does require excision. The blood supply to these aneurysmal bone cysts is robust and can result in significant bleeding at the time of surgery, so angiography is often performed to identify the blood supply and can be used to perform an embolization to limit blood loss during surgery.11

Discitis is seen in patients who present with back pain and often refusal to bear weight. The patients are usually < 10 years of age and present with various symptoms depending on their age. Patients < 3 years of age present with a failure to bear weight or a limp; those between 3 and 6 years present with abdominal signs; and older patients complain of back pain. A good clinical examination of the extremities should be performed to ensure no infection is occurring in the hips or knees. The neurologic examination is also important. X-rays will demonstrate a narrow disc space in the lower lumbar spine when discitis is present. MRI can be used to confirm the diagnosis. Successful treatment generally requires antibiotics. This is a resolving condition that does not require surgical treatment.12

Young patients with spinal cord tumors often have significant pain and walk with a very awkward pattern, oftentimes with a flexed spine to relieve their symptoms. They often present with parents suggesting that their child is walking less often and for shorter distances. A history of having accidents with bladder control is often seen. Physical examination demonstrates tenderness to palpation of the spine. Attempts at hopping on either foot are uncomfortable. A neurologic examination is critical, and urgent MRI or CT scan myelogram is necessary for evaluation. Prompt referral to a neurosurgeon is important.