Kyphoscoliosis is defined as a deviation of the normal curvature of the spine in the sagittal and coronal planes and can include a rotation of the spinal axis. Adult scoliosis is defined as a lateral deviation of more than 10 degrees in the coronal plane as measured by the Cobb angle. Lateral deviations of less than 10 degrees can be attributed to postural variation. Kyphosis and lordosis refer to the curvature of the spine in the sagittal plane. When the spine is viewed laterally, a normal degree of lordosis (posterior curvature) can be seen in both the cervical and lumbar spine ranging between 35 and 80 degrees; whereas the thoracic spine has a natural degree of kyphosis (forward curvature) generally between 30 and 50 degrees. The degree of thoracic kyphosis increases with age from 20 to 29 degrees in individuals younger than 40 years old, 53 degrees in those aged 60 to 74, and 66 degrees in patients older than 75 years.
Although kyphoscoliosis occurs most commonly in the region of the thoracolumbar spine, it can also be noted in the cervicothoracic region as well. Moderate kyphoscoliosis is a Cobb angle ranging from 25 to 100 degrees, whereas severe kyphoscoliosis is a Cobb angle greater than 100 degrees. Abnormalities in curvature and it's clinical impact are affected by the severity and location of the curvature, the number of vertebrae involved, and the degree of axial rotation. These may affect both energy consumption as well as pulmonary function.
The cause of kyphoscoliosis is multifactorial and varies based on a patient’s demographics. For simplification, causes can be classified as idiopathic, secondary, or congenital.
Given its multifactorial nature and a large number of diseases and pathologies that have a component of kyphoscoliosis, the exact epidemiology of this disease is unknown. Frequency and demographic data may be better appreciated when tailored to the primary underlying cause that may be contributing to its manifestation. A common cause of juvenile kyphoscoliosis is Scheuermann's disease which has an incidence of 0.4% to 8% in the USA, occurs more commonly in boys, and typically affects children between the ages of 13 and 16 years old.
The pathophysiology of kyphoscoliosis is dependent on the underlying etiology.
Children with kyphoscoliosis are often asymptomatic upon initial presentation and may be brought in for evaluation by a family member that is concerned about uneven shoulders or hips, prominence of part of the spine or the scapula, uneven waist, or a change in gait. In children, chronic pain or neurological signs of weakness, tingling, or loss of bowel/bladder control are rare and demands closer investigation. A thorough history should include details of growth trends, age at onset of changes, neurological changes, and psychosocial impact. In very young children, a referral to an orthopedic or neurosurgical specialist may help assuage parental concern and provide for closer follow up and earlier intervention as needed.
Older patients may present for an initial evaluation with a variety of chief complaints that may include axial back pain, concerns of cosmesis, or progressively worsening respiratory function. As in children, a thorough history should be obtained, as well as screening for changes in neurological or psychosocial health.
Physical examination should begin with a thorough inspection of height, gross anatomy/symmetry, spinal alignment, flexibility, and work of the movement. Of note, correction of kyphosis by hyperextension of the vertebrae rules out Scheuermann disease. Skin assessment for hyper/hypopigmentation, dimpling, or tufts of hair may be helpful ruling out concomitant conditions such as Neurofibromatosis, myelomeningocele, etc.
To perform Adam’s forward bend test, the patient stands facing away from the provider with their legs straight and feet shoulder-width apart. The patient then bends forward at the waist towards 90 degrees hip flexion with their arms hanging to the floor and palms placed together. An inclinometer along the thoracolumbar spine can be used to evaluate for asymmetry due to rotation or prominences. The examination also involves assessing the patient from the side while the patient transitions from lumbar flexion to upright stance and then to the lumbar extension. For children incapable of performing Adam’s forward bend test independently, a guardian or caregiver can assist in a supine or modified examination. If asymmetry is noted on examination, radiographic images may be obtained to supplement clinical findings, as often a 7 (or more) degree asymmetry noted on examination can correlate with a 20 degree spinal curve on imaging.
Assessment should also include evaluation for strength, reflexes, sensation, range of motion of all joints, limb length discrepancy, and gait analysis. Studies have shown a direct relationship between worsening kyphoscoliosis and diminished spinal extensor muscle strength. Patients may perform poorly on functional assessments of standing up from a chair, 6-minute walk test, walking speed, and the timed get up and go test.
In addition to a thorough history and physical examination, a functional assessment is a key to a complete examination. Studies have shown that the 6-minute walk test has a higher correlation to the severity of deformity than does pulmonary function testing or arterial blood gas parameters.
As previously noted, radiographic imaging assists in determining the extent of spinal curvature and is an important component of a complete evaluation. Comprehensive imaging of the spine should include upright PA and lateral views of the cervical, thoracic, and lumbar regions with an effort made to minimize any limb length discrepancy that may contribute toward excessive curvature. The Cobb angle can be utilized to assess for severity of kyphoscoliosis, risk of progression, and means of intervention. Kyphosis is defined as a Cobb angle of greater than 40 degrees in the thoracolumbar spine. On a PA radiograph of the spine, the Cobb angle is measured by the intersection of a parallel line to the most cephalad superior endplate in a particular curve, and a parallel line to the caudal most inferior endplate in a particular curve. Conventionally, perpendicular lines can then be used to compute the angle of curvature. In anatomically efficient models, a plumb line can be drawn from the body of the C7 vertebral body to the posterosuperior corner of the S1 vertebral body. When the plumb line falls anterior to the femoral head, the spine stands in a positive sagittal balance. A negative sagittal balance is noted when the plumb line falls posterior to the femoral head.
Adolescent idiopathic scoliosis is a condition in which there are structural abnormalities of the spine in the coronal, sagittal, and axial planes. In 1983, The King classification was established to describe the five thoracic curve types and the spinal arthrodesis recommended for specific vertebral levels. As surgical techniques and equipment evolved, limitations of The King Classification were brought to light and the system fell out of favor. The Lenke Classification was established in 2001 and emphasizes the sagittal plane in addition to the coronal plane. The system is organized according to three main parameters: curve type (1-6), sagittal thoracic modifier (-, N, or +), a lumbar spine modifier (A, B, C). Further details on these classifications and their criteria are beyond the scope of this text.
If further investigation is warranted, a bone mineral density scan or an MRI may facilitate the transition for possible surgical intervention. Studies have shown a relationship between increased Cobb angle and poor performance on pulmonary function testing (PFT).
Structural abnormalities frequently affect pulmonary function, resulting in reduced exercise tolerance and ventilation. The examination may reveal asymmetric chest expansion and poor inspiratory capacity secondary to restrictive changes. Pulmonary function testing may reveal a reduction in functional residual capacity (FRC), Forced vital capacity (FVC), and FEV1. The development of cor pulmonale carries a poor prognosis and a higher risk of mortality. An echocardiogram may assist in assessing pulmonary arterial hypertension.
The preferred treatment method is dependent on the underlying cause, age, the severity of the deformity, and the absence or presence of neurologic deficits. Treatment is typically initiated in a conservative manner and may progress to the point that surgical intervention is required. In younger patients, kyphoscoliosis may be an incidental finding with little to no effect on function. However, if pathology is significant enough to cause pain, neurologic changes, concerns of cosmesis, or concerns of progression, then further testing and referral to a specialist are recommended.
In patients recommended non-operative management, treatment can include:
In patients experiencing neurological changes, operative management may be warranted. This population is particularly sensitive to peri-operative medication and side effects due to functional limitations and prolonged immobilization. Surgical correction for a hypermobile spine can include an arthrodesis and the use of hardware such as rods, wires, and screws, whereas osteotomies can be used to relieve rigid spines. In patients without neurological compromise, procedures such as vertebroplasty and kyphoplasty have recently been gaining favor as a means for vertebral augmentation. Operative management may be indicated in those patients who have failed conservative therapy, experience intractable pain, have an onset of neurologic changes, or those that have persistent progression despite bracing.
Usually curves greater than 45 and 50 degrees are surgically managed.
The two methods of surgical intervention are:
The various correction osteotomies for kyphosis include:
Post-operatively, a patient's course should be closely monitored to optimize pain control and assess for any neurological changes. Surgery is not typically recommended in elderly patients given their high-risk profile and poor bone health, however, it can be carefully considered as an option for those patients affected by significant malformation.
Kyphoscoliosis may be idiopathic and be noted in otherwise healthy individuals. In children, the finding may be mild and should be followed over time to assess for curve progression. Rather than considering a differential for kyphoscoliosis, it may be more beneficial to consider the underlying cause contributing to its presentation or the constellation of symptoms in which it presents. As mentioned above, skin changes may play a role in identifying concomitant conditions such as neurofibromatosis and myelomeningocele. Some other conditions that should be considered include:
The prognosis for kyphoscoliosis is guarded and is dependent on the underlying etiology contributing to disease progression. Kyphoscoliosis complicated by pulmonary hypertension is associated with high mortality.
Kyphoscoliosis increases the risk of all-cause mortality.
Patients should be educated on how these structural changes may result in physical limitations, increased risk of falls, diminished strength, diminished pulmonary function, and increased mortality.
The management of kyphoscoliosis is challenging and complex. To derive good outcomes, the goal is to minimize structural abnormality and progression and optimize functional capacity and independence. Initially, management of kyphoscoliosis is principally by the primary provider and likely includes conservative management techniques comprised of observation, physical therapy, pain management, bracing, etc. However, in a case resulting in rapid progression, refractory pain, or neurologic compromise, referral to a surgeon or specialist is warranted and operative management may be indicated. Thus, an interprofessional team that provides a holistic, unified, and shared decision-making approach can optimize patient care and help maintain functional ability. This team can include but is not limited to: the patient, family, primary provider, therapists, surgeons, orthotists, pulmonologists, and/or neurologists.
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