Groningen, The Netherlands
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Lateral Curvature(s) of the Back
Complaint: curved back.
Assessment: assessment shows one or two lateral curvatures in the spinal column.
Ask the patient to bend forward until the trunk is horizontal, hanging the arms down loosely with the palms facing each other (forward bending test). If the lateral curvature disappears and there is no hump, then there is a non-structural scoliosis. In a structural scoliosis the curve remains visible during the forward bending test, and any hump will also be more clearly visible (Fig. 3.1).
(a) Right convex thoracic scoliosis. (b) Nonstructural scoliosis. In the forward bending test there is no hump: on the contrary, the scoliosis disappears entirely. (c) Structural scoliosis. In the forward bending test a thoracic prominence (rib hump) is more visible
postural scoliosis compensatory scoliosis
congenital scoliosis idiopathic scoliosis
infantile idiopathic scoliosisjuvenile idiopathic scoliosisadolescent idiopathic scoliosis
Explanatory note: scoliosis. The anteroposterior projection of a normal spinal column is straight in the frontal plane. If the lateral curvature measured with the Cobb method is 10° or less (Fig. 3.2), we speak of a physiological asymmetry. A scoliosis entails one or more lateral curvatures of the spinal column with a Cobb angle greater than 10°.
(a) The degree of lateral curvature (scoliosis) is measured according to the Cobb method, by drawing lines parallel to the superior endplate in the top vertebra and the inferior endplate of the lowest vertebra in the curvature. Then drawing perpendicular lines to these lines. The angle between these perpendicular lines, known as the Cobb angle (*), indicates the degree of the lateral curvature in degrees. (b) Scoliosis means one or more lateral curvatures of the spinal column, where the lateral curvature measured according to the Cobb method is 10° or more
In a nonstructural scoliosis no gibbus is observable in the forward bending test. Manifestations of a non-structural scoliosis are postural and compensatory scoliosis.
If present, it is usually seen at the end of the first decade of life. There is a slight, usually left convex thoracic curve. It disappears when the child lies down and can be actively corrected when standing. When bending forwards there is no hump, and the lateral curve disappears completely (Fig. 3.3). This curvature never becomes structural. The lateral curvature disappears spontaneously as the child grows.
(a) Postural scoliosis, left convex thoracolumbar curvature. (b) The scoliosis disappears during the forward bending test
A difference in leg length causes a compensatory scoliosis only when standing. The convex side is on the side of the short leg. There is no torsional component. The scoliosis disappears when sitting, when lying down, also after correcting the difference in leg length, and also during the forward bending test (Fig. 3.4). This curvature never becomes structural either.
(a) Compensatory scoliosis, the convex side points to the leg that is too short. (b) The same patient as in Fig. 3.4a. The scoliosis disappears after correction of the leg length difference
Manifestations of a structural scoliosis are congenital, idiopathic and neuromuscular.
This scoliosis occurs due to abnormal vertebrae such as hemivertebrae and unilateral nonsegmented bony connections (Fig. 3.5). In 75 % of these cases there is a progressive scoliosis of 5° per year, in 14 % there is slight progression, and only 11 % are non-progressive (Table 3.1). The abnormality occurs usually at the level of the thoracic spine. On physical assessment the scoliosis cannot be distinguished from a progressive infantile or juvenile idiopathic scoliosis (see below). Radiological assessment provides information. Children with a congenital scoliosis often have other abnormalities too (Table 3.2). The most dangerous accompanying abnormalities are congenital cardiac anomalies (5–10 %) and abnormalities of the urogenital system (20 %). In 10 % of cases there is a spinal dysraphia (Table 3.2).
Congenital scoliosis. (a) Hemivertebra. (b) Non-segmented bony connections
Progression of the different scolioses
75 % strongly progressive
14 % lightly progressive
15 % progressive
90 % progressive, especially after age 10
Depending on age and size of the curve at the time of discovery. See Table 3.2.
Nearly always progressive, pelvic obliquity
Abnormalities associated with a congenital scoliosis
Abnormalities of the chest wall
Absence of lower extremity or parts of it
Congenital heart abnormalities 5–10 %a
Congenital urinary tract abnormalities 20 %a
Cranial or head abnormalities
Leg length inequality
Rib anomalies (extra or absent ribs, deformed or fused ribs)
Spina bifida occulta
Spinal dysraphia 10 % (spina bifida, diastematomyelia, tethered cord or lumbosacral lipoma)
These are developmental disorders in the median line of the back. The most common is spina bifida. Diastematomyelia (Fig. 3.6), tethered cord (Fig. 3.7) and intraspinal lumbosacral lipoma (Figs. 3.8 and 3.9) are also spinal dysraphias. In half of all cases physical assessment shows signs that point to a spinal dysraphia. These can be skin abnormalities in the lower back in the midline such as enhanced hair growth (lumbar hypertrichosis), pigmented or depigmented naevi, port-wine stains, a sacral dimple, (Fig. 3.10) a fistula or a subcutaneous lipoma.
Diastematomyelia. A bony, cartilaginous or fibrous partition in the middle of the spinal canal
Tethered cord: the distal part of the medullary cone is attached to the distal end of the spinal cord canal with a fibrous strand (terminal filum). In newborns the distal end of the spinal cord (medullary cone) is at the level of L3. During growth the medullary cone ends up cranially. In adults at level LI-L2. In a tethered cord the medullary cone does not shift cranially during growth
Lumbosacral lipoma: an extraspinally located part of the lumbosacral lipoma is often connected with an intraspinal part. This fixes the conus at a lower level and neurological symptoms can also occur
Hypertrichosis and a fistula
(Latin for split spine). This is a birth defect with an incomplete closing of the spine. There are three types: spina bifida occulta, meningocele and myelomeningocele. The most common location is the lumber spine. The spina bifida occulta has no or only skin signs at physical examination. A meningocele causes a sac of fluid at the gap in the spine. A myelomeningocele is the most severe form associated with hydrocephalus, tethered cord, anomalies of the lower extremities, walking problems and problems with bladder and bowel control.
In a diastematomyelia (diastema is Greek for in-between space) there is a bony, cartilaginous or fibrous partition in the middle of the spinal canal (Fig. 3.6). As a result of traction on the spinal cord, which is fixed at the position of the partition in the spinal canal, pain and progressive neurological symptoms such as urinary problems, gait disorders and foot deformities occur in growing children.
Tethered cord may also described as an attached low medullary cone or a fixed myelum. The lowest cone-shaped end of the spinal cord (medullary cone) normally lies at the level of L3 in newborns and at the L1 or L2 in adults (Fig. 3.7). The distal end of the medullary cone is attached to the distal end of the spinal cord canal by a fibrous band (terminal filum). During growth the lower end of the myelum (spinal cord) does not shift cranially in a tethered cord. Adhesion of the cone often also occurs and the the filum is terminally shortened. During growth progressive neurological symptoms can develop as a result of traction on the spinal cord.
VATER or VACTERL association
In a congenital scoliosis one should also consider a VACTERL association (V stands for vertebral abnormalities, A for anal atresia, C for cardiac anomalies, T and E for tracheoesophageal fistula, R for renal anomalies such as urethral atresia and renal agenesis, and L for limb defects such as radial aplasia1).
This is a scoliosis in which the cause is unknown. The curvature may consist of a single or double curvature (S-shaped). The position and direction of the lateral curvature remains unchanged in the course of time, but if the anomaly increases several additional vertebrae may become involved. In single curvatures compensatory curvatures tend to appear, so that the spinal column remains in balance. If there is a loss of balance, misalignment can be assessed using a perpendicular line. Compensatory curves are in principle not structural but can become so with the passage of time.
In an idiopathic scoliosis there is a torsion abnormality in the transverse plane, in addition to an abnormality in the frontal plane. At the level of the thorax the torsion deformity manifests itself with prominence of the ribs on the convex side of the curve, known as a rib hump (Fig. 3.11). The torsion at the lumbar level causes a unilateral prominence of the back muscles. Idiopathic scoliosis is always accompanied by a torsion in the spinal column. The scoliosis can be classified according to the position of the lateral curves (Fig. 3.12). Idiopathic scoliosis affects 2–3 % of children, especially starting from 10 years of age. There is a strong congenital component. A prevalence of scoliosis is found in 18 % among first-degree relatives.
(a) Right thoracic scoliosis. (b) A thoracic prominence of the ribs (rib hump) is clearly visible during the forward bending test. (c) A rib hump occurs because an idiopathic scoliosis is always accompanied by a torsion in the spinal column. At the chest level this manifests itself by a bulge in the ribs on the convex side, which becomes clearer when bending over forward. Upon physical assessment the scoliosis seems less severe than on the X-rays because the spinous processes rotate toward the concave side in the arch
The scoliosis can be classified on the basis of the localization of the lateral single or double curvature
A classification has been made with the following three age groups because idiopathic scoliosis can occur at different ages:
infantile idiopathic scoliosis from ages 0–3
juvenile idiopathic scoliosis from 3 to about 10 years of age
adolescent idiopathic starting from around the age of 10
Some scoliosis experts doubt whether juvenile scoliosis consists of a separate group and claim that this scoliosis may be a late-diagnosis infantile or an early-diagnosis adolescent scoliosis. In recent years these groups have been put into two groups, early onset and late onset. Some set the boundary at 5 years, therefore merging juvenile and adolescent scolioses. Other experts set the boundary at age of 8, merging infantile and juvenile scolioses.
Infantile idiopathic scoliosis (ages 0–3)
This is rare and comprises 1 % of all scolioses. It affects boys more often than girls (3:2). The lateral curvature is convex to the left and thoracically localized (Fig. 3.13). Accompanying anomalies may be facial and cranial asymmetry (plagiocephaly), torticollis or developmental dysplasia of the hip. In 85 % of cases the curvature fortunately disappears before the first year of age. Spontaneous disappearance of the curvature never occurs if the curvature is more than 35°. In 15 % of cases the curvature does not disappear spontaneously and a severe deformity is the result (Table 3.1). It is important to make a distinction between nonprogressive and progressive infantile scoliosis. This is best achieved by requesting an anteroposterior X-ray of the spinal column and measuring the ribvertebral angle difference at the top of the curvature (RVAD or Metha angle). The angle between the spinal column and the rib, on both the left and the right sides, is measured. If the difference is more than 20° we are dealing with a progressive infantile idiopathic scoliosis in 90 % of cases. In 10 % of cases the scoliosis will disappear spontaneously (Fig. 3.14). If the RVAD is less than 20° spontaneous disappearance occurs in 90 % and in the remaining 10 % will become progressive. It is prudent to follow-up the child every 4 months radiologically from age 1 to age 2, to determine non-progression or progression.
Infant with infantile idiopathic scoliosis, left convex lateral curvature
Rib-vertebral angle difference (RVAD or Metha angle). At the top of the curve the angle was measured between the spinal column and the rib, on both the left and the right side. If the difference is more than 20°, then we are most likely dealing with a progressive idiopathic scoliosis. In this case it is 85°−54° = 31°, therefore most likely progressive (Redrawn from: Metha MH. Rip-vertebra angle in the early diagnosis between revolving and progressive infantile scoliosis. J Bone Joint Surg Br. 1972;54-B:230–43)
Juvenile idiopathic scoliosis (ages 3–10)
This involves 10–20 % of all idiopathic scolioses. This type of scoliosis is equally common among boys and girls. It usually involves a right convex thoracic scoliosis (Fig. 3.15) or a double curvature: thoracic convex to the right and lumbar convex to the left. In girls between 6 and 10 years of age the curvatures are more severe than in boys. If there is a convex thoracic curve to the left at this age, intraspinal pathology should be ruled out with an MRI. This also applies to curvatures of more than 20° because in 25 % of such cases other spinal pathology will be found. The curvatures never disappear spontaneously as is the case in infantile idiopathic scolosis. About 90 % of juvenile lateral curvatures, are progressive and require treatment especially after 10 years of age (Table 3.1). If the rib-vertebral angle difference (RVAD) is less than 10°, there is generally no progression in the curvature. Progressive curvatures are mostly those with a rib-vertebral angle difference (RVAD) greater than 10°. This is particular so in the case of curvatures with an apex of the curvature between T8 and T10. This is also the case when the angle is more than 45° at the first consultation with a thoracic kyphosis of less than 20° (normal range 20–50°). Juvenile idiopathic scoliosis must be checked radiologically every 4 months to see if there is any progression.
(a) Five-year-old girl with juvenile idiopathic scoliosis. The thoracic curvature is convex to the right. (b, c) Untreated juvenile idiopathic scoliosis
Adolescent idiopathic scoliosis (from the age of 10)
Scoliosis in adolescence is the most common type. In the age group between 10 and 15 years, 2 % have a lateral curvature between 10° and 20°, with a male:female ratio of 1:2. A curvature between 20° and 30° occurs in 0.3–0.5 % in this age group, with a male:female ratio of 1:5. A curvature of more than 30° occurs in 0.1–0.3 % of adolescents, with a male:female ratio of 1:10. The most common curvature is a right convex thoracic curvature (Fig. 3.16), followed by a double curvature thoracic to the right and lumbar to the left and in the third place a curvature with a left or right convex lumbar scoliosis. If there is a left convex thoracic curvature at this age, intraspinal pathology must be ruled out with an MRI. The first complaint is not always the lateral curvature of the spinal column but an elevated position of the shoulder with a crease on the concave side of the body or that one breast is positioned slightly more anteriorly than the other. Asymmetry of the air silhouettes between arms and trunk is often noticeable. The lateral curve may not be progressive. In some cases it is stationary. As a rule of thumb, the following applies: the greater the curvature at the moment it is identified, the greater the chances are of progression, especially in children between the ages of 10 and 12 (Table 3.3). On average, one can say that in children between the ages of 10 and 15, with curvatures less than 20° the chances of progression are 20 %, and in curvatures more than 20° the chances of progression are 70 %. It is impossible to predict which curvatures will remain stationary and which won’t, although thoracic curvatures have greater chances of progression than lumbar ones. Curvatures less than 30° increase little during the rest of their lives after the children have grown up. A curvature between 30 and 50° increases 10–15 % after growth. However, if the scoliosis is more than 50° the lateral curvature increases due to premature degenerative changes in the intervertebral joints and the intervertebral discs. At the thoracic level this increase can even amount to 30° at an older age, with an average increase of 1° per year. In lumbar curvatures there is less of an increase, but at this level more pain is experienced with these curvatures than with thoracic curvatures. In curvatures less than 50° the chances of back problems is not greater than in individuals without a scoliosis. Cardiopulmonary problems only occur with curvatures more than 60–70°. A reduced life expectancy is only the case with curvatures of 100° or more.
(a) Adolescent idiopathic scoliosis with a right convex thoracic curvature. (b) A rib hump is visible during the forward bending test
Progression of an idiopathic adolescent scoliosis related to the size of the lateral curvature and age at the first X-ray
Size of the curve
Chances of progression at the age of
Supplementary assessment: in a structural scoliosis, an anteroposterior and lateral X-ray of the entire thoracic and lumbar spine should be made. In addition an anteroposterior X-ray should be taken of the whole vertebral column (a so-called scoliosis X-ray). In idiopathic scoliosis, in addition to the lateral curvature there is also a torsion of the spinal column. This torsion can be detected based on the position of the pedicles (Fig. 3.17). In single curvatures a compensatory curvature may also be present. Lateral flexion images of the spinal column to the left and to the right give insight into the suppleness of the curve. A secondary or compensatory curvature tends to be completely straight (Fig. 3.18). If there is a congenital scoliosis, a CT-scan and MRI should also be taken. In infantile and juvenile scoliosis the rib-vertebral angle difference (RVAD) can be calculated (Fig. 3.14). A MRI should be requested to rule out intraspinal pathology if a left convex thoracic scoliosis is present in juvenile or adolescent patients. The same applies to juvenile scoliosis with a Cobb angle greater than 20°.
Primary care treatment: a lateral curvature due to a postural deviation will disappear spontaneously. A compensatory scoliosis due to a difference in leg length can be corrected with an insole up to 1½ cm or a complete sole elevation up to 2 cm (for larger differences in leg length, see Chap. 15).
When to refer: a congenital scoliosis should always be referred to a pediatrician and an orthopedic surgeon specialized in spinal column surgery. In the case of an infantile idiopathic scoliosis that is still present at 1 year of age and a juvenile and adolescent scoliosis with a curve measured according to Cobb of 10° or more should be referred to an orthopedic surgeon. A compensatory scoliosis resulting from a difference in leg length of more than 2 cm is also an indication for referral, in order to assess whether operative correction of the leg length difference is required.
Secondary care treatment: congenital scoliosis. In a progressive congenital scoliosis a spondylodesis, combined if necessary with a hemivertebra resection at the level of the congenital abnormalities, is performed as soon as possible. A non-progressive congenital scoliosis should be monitored every 4 months for a few years, to be on the safe side.
In a structural scoliosis there is torsion of the spinal column in addition to a lateral curvature. This rotation can be observed based on the position of the pedicles on an anteroposterior X-ray of the spinal column
(a) In single curvatures a compensatory curvature can occur. Lateral flexion images of the spinal column to the left (b) and (c) to the right give insight into the suppleness of the arch. (b) The secondary or compensatory curvature (in this case lumbar) tends to completely corrected. (c) The primary or structural thoracic curvature cannot be corrected. The secondary lumbar curvature increases
Scolioses with a lateral curve between 10° and 20° are checked once every 4 months by an experienced practitioner to assess possible progression in the lateral curve. If these check-ups show that the curvature is increasing, or if the practitioner has doubts about it, new X-rays must be taken.
Infantile idiopathic scoliosis
In the case of progression a plaster cast or orthosis treatment is indicated. In the case of progression one should delay carrying out a spondylodesis if possible until after 10 years of age.
Juvenile idiopathic scoliosis
This is usually progressive, especially after 10 years of age. The first step is to treat with a orthosis. The majority of these scolioses can undergo a spondylodesis after the age of 10.
Adolescent idiopathic scoliosis
Progression in an adolescent idiopathic scoliosis usually occurs in early puberty, when armpit and pubic hair become manifest and external sexual characteristics develop, such as growth of the breasts and the testicles. In a progression of more than 5° a orthosis will be prescribed. The orthosis should be worn day and night, preferably for 23 h a day. The scoliosis and the orthosis are checked every 4 months. This treatment is continued until growth is complete. In girls growth stops at around the age of 14, in boys around the age of 16½. One gets and impression when growth is completed by measuring body height at each checkup. If the body height remains constant on two consecutive checkups, in girls aged 12½ or older and in boys aged 14½ or older, a skeletal image of the left hand can be made to see if growth has really stopped (Fig. 3.19).
Hand skeleton X-ray. Skeletal age is determined based on an X-ray of the left hand. It is possible to establish the skeletal age by comparing the size and shape of the epiphyseal growth plates according to the atlas of Greulich and Pyle. The skeletal age based on this X-ray is 12½ years (Redrawn from: Greulich WT, Pyle SI. Radiographic atlas of skeletal development of the hand and wrist. 2nd ed. Stanford University Press; 1959)
Another method is to establish the Risser stage, where the degree of ossification of the apophysis of the iliac crests is determined on X-ray (Fig. 3.20). If on the first appointment a curvature between 20° and 50° is found then orthosis treatment should be started immediately. A orthosis will not improve the scoliotic angle. The orthosis does prevent progression in about 80 % of cases.
The Risser sign determines the degree of osseous maturity of the apophysis on an anteroposterior X-ray of the pelvis. The drawing shows stages I-IV on the right iliac crest, with a constant 25 % increase of ossification per stage. The ossification begins on the interior side of the pelvis. Risser I means that 25 % of the pelvis is ossified; this indicates the beginning of puberty. Risser IV means 100 % ossification, but no full fusion yet. Risser V (left pelvic half) shows full fusion of the apophysis with the ilium (Redrawn from: Risser JC. The iliac apophysis, an invaluable sign in the management of scoliosis. Clin Orthop Relat Res. 1958;11:111–9)
The most common orthosis used is the TLSO (thoracolumbosacral orthosis) or a Boston orthosis (Fig. 3.21). This orthosis is effective for curvatures smaller than 35°. An alternative to the Boston orthosis is the Triac orthosis (Fig. 3.21). Both can only be used when the apex of the curvature lies at T8 or more distally. An extension to the chin is generally needed in curvatures where the apex lies cranially to T8. This is the case with the Milwaukee orthosis (Fig. 3.22). However this orthosis tends to be poorly tolerated. Besides this problems may occur with the mandibular heads. Fortunately, curvatures with an apex cranial to T8 are not common. The orthosis should be worn daily 22–23 h, and removed for sports.
(a) Boston orthosis. (b) The orthosis is not noticeable when dressed. (c) Triac scoliosis orthosis. (d) This orthosis allows movements, in this case sideways to the right
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