As these conditions often involve newborns, a calm and relaxed environment is preferred to best assess the full extent of the child’s cervical motion. A crying or upset baby may resist the examiner when attempting to range the child’s neck. It can be incredibly hard to determine if a tight SCM is
due to fibrosis or is merely a normal muscle that is contracted by an irritated infant. Palpation of the neck can also give clues as to the cause. In CMT, a knot may be palpated in the SCM muscle (Figure 11.6).

It is important to remember that the absence of the mass does not rule out a muscular cause for the torticollis. A good examination of the throat and neck is important to detect swelling in the neck or palpable lymph nodes, which might be a clue of an infectious cause for the torticollis as in the case of Grisel syndrome.

As children get older, it can be easier to examine the child and detect the tightened SCM (Figure 11.7).

It is important to note the direction in which the head is tilted. In CMT, the head always tilts toward the affected side and is rotated away from the involved SCM (Figure 11.8).

The next step is to determine the range of motion of the neck, which may not be possible in late onset torticollis, as a result of pain from inflammation or trauma. When examining an infant, place the parent or favorite toy opposite the side of the resting neck position. The child will want to turn toward the parent or object, which may help determine the amount of rotation opposite the deformity. Serial measurements at each visit of the amount of motion in each plane are important to determine the amount of improvement and ultimately the success of treatment.

Older patients may be able to follow directions allowing determination of active range of motion but in the younger patients, only a passive examination of motion will be possible. Cervical motion is measured in terms of neck flexion, extension, left and right rotation, and side-to-side bending (Figure 11.9).

Depending on the age of the patient, a more thorough neurological examination should be performed as well. Deficits in upper and lower extremity strength and sensory examinations can point to an underlying neurological cause for the torticollis. It is important to remember that late-onset torticollis can be the initial finding for intraspinal neoplasia. Ocular and visual testing may be appropriate in certain settings as well (Figure 11.10).

Children with congenital cervical spine fusions usually do not have classic rotation in one direction with tilt in the other. More often, they have tilt with symmetric limitation of motion without any muscle tightness. When evaluating a child with congenital cervical fusions, it is
important to remember the association with Klippel-Feil deformity as well as the possibility of vertebral abnormalities in the thoracic and lumbar spine. These children can occasionally have an associated small, elevated scapula (Sprengel anomaly) which then limits their ability to fully abduct their arm due to abnormal scapula-thoracic mechanics (Figure 11.11).

As mentioned, infants with a congenital torticollis can be difficult to examine and it can be challenging to confirm that it is a tight SCM from CMT or is a result of congenital cervical fusions. A tight SCM can respond to treatment with physical therapy where head tilt from bony fusions do not. It is a good idea to order cervical spine X-rays as well as a lateral skull X-ray. The later film is needed to properly visualize the upper cervical spine.

In later onset torticollis, the differential diagnosis can include neoplasia in the brain or spinal cord and AARD from infection or trauma. X-rays are ordered to look for fractures, displacement, and
soft tissue swelling. Laboratory markers for infection may be needed if an infectious cause is considered. Advanced imaging such as an MRI is needed if neoplasia is suspected. Dynamic CT scan can be obtained to document rotatory subluxation (Figure 11.12).

Treatment depends on the cause of the torticollis. For CMT, a program of physical therapy is initially started to stretch the tightened SCM (Figure 11.7). Occasionally, surgical release of the SCM is needed if torticollis does not resolve with more conservative methods (Figure 11.13).

Treatment for Klippel-Feil syndrome and congenital bony abnormalities is often observation. Surgical treatment is rarely needed and only for progressive deformities. In terms of AARD, supportive treatment with a soft collar is sometimes all that is needed in the acute presentation. More chronic or resistive presentations may result in surgical stabilization (Figure 11.5).

Adolescents with scoliosis do not usually seek medical evaluation because of back discomfort but rather because of some physical aspect of their deformity, such as a high shoulder, one-sided prominence of a scapula or breast, elevated or protuberant iliac crest, or asymmetry in flank creases and the trunk (Figure 11.15). Except for being noticed personally by the adolescent, these findings are often first appreciated during school screening programs for scoliosis or during back-to-school and sports physical examinations by the family physician.

Similar to the incidence of back pain in adolescents without scoliosis, back pain can occur in individuals with idiopathic scoliosis. Nearly 32% of adolescents with idiopathic scoliosis complain of back discomfort at some point (23% at initial evaluation and 9% during the period of observation).¹ When an adolescent with presumed idiopathic scoliosis has back pain, a careful history should be obtained, a thorough physical examination performed, and plain radiographs ordered. If findings on this initial evaluation are normal, a diagnosis of idiopathic scoliosis can be made, the scoliosis can be treated appropriately, and nonsurgical treatment of the back pain can be initiated. It is not necessary to perform extensive diagnostic studies in every adolescent with scoliosis and occasional mild back pain. Simply educating the family that just because their child has scoliosis does not mean they are related may be all that is required. Further evaluation with an MRI of the spine and spinal cord may be useful if the patient’s back pain becomes a dominant feature (night pain, pain at rest, pain that radiates down the leg) and significantly restricts normal activities, if they have systemic changes in health (fatigue, weight loss, fever), or if they have findings on physical examination that are abnormal (foot deformity, abnormal reflexes, spine imbalance, weakness).

While respiratory symptoms are uncommon in patients with adolescent idiopathic scoliosis (AIS), pulmonary assessments have identified subclinical yet measurable mild restrictive lung disease in one-third of patients with idiopathic scoliosis. When the breathing kinematics of the chest wall and spine is evaluated, individuals with AIS have decreased motion in comparison to healthy individuals. The effect on lung function is dependent on the magnitude of deformity. Studies have shown that clinically significant cardiopulmonary compromise does not usually occur until the magnitude of the curve approaches 80° to 100°. When thoracic lordosis (exceeding 20°) significantly narrows the anteroposterior dimensions of the chest, vital capacity becomes less than 45%.

In summary, AIS has a small measurable effect on lung function that patients do not notice in all but the largest of curves. Problems with respiration do not exist unless the curves reach 100° (Figure 11.15) or unless there is significant pulmonary comorbid conditions.

Neurologic deficits are also rare in individuals with AIS, yet a detailed physical examination can yield findings that can identify structural spinal cord abnormalities that can be present in what appears to be idiopathic scoliosis in an apparently normal child. An adolescent with a large Chiari malformation (Figure 11.16) may describe symptoms such as persistent neck pain, frequent headaches, difficulty swallowing, ataxia, or weakness. Unilateral foot deformities may be associated with tethering of the spinal cord (Figure 11.17). In addition, the convexity of thoracic curves in AIS is normally directed to the right; abnormal left thoracic curves are more common in those with an underlying syrinx (Figure 11.18). In addition, if any neurologic deficits are found on examination, appropriate imaging of the neural axis is undertaken.

Physical examination of an adolescent with idiopathic scoliosis should be performed with the patient properly draped. The patient may be dressed in underpants and an examination gown open at the back. The patient’s entire back, including the shoulders and iliac crests, must be visible. The skin is inspected closely for abnormalities such as café-au-lait spots (Figure 11.19).

Other cutaneous aspects include midline hemangiomas, hair tufts, and dimpling in the lumbosacral region. Any of these surface findings may indicate the presence of an underlying spinal cord abnormality such as a tethered cord or diastematomyelia (this term describes a spinal cord that is split by fibrous, cartilaginous, or bony tissue). The spinous processes are palpated from the cervical region to the sacrum for any deficiencies or areas of discomfort. Occasionally, absence of a spinous process is noted, which usually corresponds to spina bifida occulta seen on a spinal radiograph.

With the patient standing, the examiner should determine whether the iliac crests are level (Figure 11.20). If they are not, a lower limb length discrepancy is likely, and which can be quantified by placing measured blocks under the short extremity until the iliac crests are level. Leg length discrepancy can be responsible for the appearance of lumbar scoliosis, and the condition must not be overlooked as timely treatment is available for those children who are growing. In these cases, one can confirm that the scoliosis is postural and compensatory by having the child stand on blocks

or simply sit on the examination table and examining the now straightened lumbar spine from the back.