CHAPTER
72
Natural History Including
Measures of Maturity
Treating scoliosis implies we can improve upon its natural history.
Because the typical goal in scoliosis treatment is to prevent future
problems, a detailed understanding of its natural history is very
important to clinicians. Our understanding of idiopathic scoliosis
behavior comes from a limited group of important studies, few of
which use validated functional outcome measurements. This,
unfortunately, leaves us bereft of some important information.
We are also only beginning to define the natural history of idio-
pathic scoliosis during growth. This chapter delineates what these
studies show us about the natural history of idiopathic scoliosis in
terms of the curve progression and patient function.
PREVALENCE AND INCIDENCE
Small curves and mild spinal asymmetries such as scapular
winging, shoulder elevation, or rib prominence are common
and found in 3% to 15% adolescents. Larger curves are much
rarer and males have a much lower prevalence than females
(see Table 72.1). Curves greater than 40 occur less than 1 in
1000. In a recent review, Dolan and Weinstein14 summarized
data from the Center for Disease Control (CDC) and the
Agency for Healthcare Research and quality (AHRQ) identify-
ing 602,884 private physician office visits codes for idiopathic
scoliosis in 19958 and more than 4500 surgeries for adolescent
idiopathic scoliosis in 2000.1
TYPES OF IDIOPATHIC SCOLIOSIS
Idiopathic scoliosis types are currently defined by the age of
onset and the specific curve pattern.
AGE OF ONSET
Idiopathic scoliosis is separated by age of onset into infantile,
juvenile, and adolescent age groups with infantile scoliosis
defined as that developing from birth to 3 years, juvenile from
ages 4 through 9, and adolescent from 10 years to maturity.
Unfortunately, distinguishing curve onset from discovery can
be difficult or impossible. A separate classification system
LWBK836_Ch72_p711-720.indd 711
James O. Sanders
groups scoliosis into early onset for ages birth to 5 years and
late onset for those older than 5 years, based on reasoning that
the pulmonary risk is less for those with late onset. However,
even juvenile curves have a higher mortality rate than adoles-
cent curves. Central axis abnormalities exist in approximately
20% of both infantile and juvenile scoliosis patients making
spinal magnetic resonance imaging important, which is not
true for adolescent idiopathic scoliosis.13 So, despite its limita-
tions, classification as infantile, juvenile, and adolescent scolio-
sis remains useful.
PATTERN
Curves are described by their location, apical direction, and
Cobb angle. Typically, although inconsistently, nonoperated
scoliosis is described for each individual curve separately. Sev-
eral authors have developed specific curve type classifications,
but none are universally accepted for nonoperative scoliosis.
Operative curve classifications rely to some degree on curve
flexibility from bending or traction radiographs, which are
rarely available for nonoperative curves. The King classification
was designed prior to the era of segmental instrumentation to
determine the feasibility of a selective thoracic fusion, whereas
the Lenke classification18 attempts to separate which of multi-
ple curves require instrumentation and fusion. The reliability
of distinguishing the various curve patterns is problematic, but
it remains important because differing curve patterns behave
differently through growth. We have used a modified Lenke
classification and found that occasionally curves change pat-
tern during growth,29 such as the development of a secondary
lumbar curve from an initial single thoracic curve pattern.
Three-dimensional classifications hold significant promise
but are limited to a few research centers with sophisticated
imaging and software. Identifying curve patterns through fuzzy
clustering techniques from three-dimensional images15 looks
promising but must await common use of three-dimensional
imaging and complex calculations before it can be widely
used. Until then, a modified Lenke classification seems useful
provided the difficulty of describing a complex three dimen-
sional deformity from a single posteroanterior radiograph is
understood.
711
8/17/11 11:14:51 AM
 712 Section VII Idiopathic Scoliosis

growth, the start and end of the growth spurt, and progression
Prevalence of Scoliosis Based
TABLE 72.1 during adulthood. Ideally, functional end points are preferred,
on Curve Magnitude such as poor appearance, pain, or pulmonary problems, but
these points are currently nebulous. Scoliosis progression
Cobb Angle Female:Male Ratio Prevalence (%)
occurs in four basic phases: infantilerapid, juvenileslow,
5 1:1 for 6 to 10 curves 4.5 adolescentrapid, and matureslow (see Fig. 72.1). When
10 1.4 to 2.1 2 to 3 plotted over time, curves can increase quite quickly in the rap-
20 5.4:1 0.3 to 0.5 idly growing infant. Following this, there is typically a long
30 10:1 0.1 to 0.3 latent period with little curve progression during the juvenile
40 0.1 slow phase. Progressive curves significantly change their behav-
ior during the adolescent growth spurt. During this phase,
Data from Weinstein41 and Rogala et al.42
curves separate into rapid, moderate, and low rates of progres-
sion. Curve progression then significantly slows or halts for the
mature phase at the completion of growth. Time relative to this
CURVE EVOLUTIONPROGRESSION curve acceleration phase (CAP) may be defined in months
DURING IMMATURITY before or after the inflection point, CAP 0. For example, CAP
-6 represents 6 months before the acceleration and CAP 12
The relationship between growth and scoliosis progression was represents 1 year after the acceleration.
known to the pioneers5,6 of scoliosis treatment, but Duval-
Beaupere43 clearly demonstrated the close relationship between
height increase and scoliosis progression. She showed that INFANTILE
curve progression increases markedly at the adolescent growth
spurt for both idiopathic and neuromuscular curves. Since this Infantile curves may be either resolving or progressive, with
also holds true for congenital scoliosis,7 it appears to be inde- resolving curves far more common. The ribvertebral angle of
pendent of etiology. Mehta,21 the difference formed by the apical vertebral bodys
Most older scoliosis studies used definitions of curve pro- perpendicular and the two rib heads (Fig. 72.2), and the rib
gression such as 5 or 6. Since curves rapidly progress during a phase are important prognostically. The rib phase is classified
very limited phase of growth, it is cautioned against making as phase 1 if the convex rib at the apex does not overlap the
decision based on these definitions and we recommend using vertebral body and phase 2 if it does overlap (Fig. 72.3). In
physiological end points such as slowing of the rapid infantile progressive curves, the ribvertebra angle difference (RVAD)

70 20
Juvenile Adolescent Early Mature
18
60
eCAP 0
PHV eCAP +6 16
TRC 2 Tanner eCAP +12
50 eCAP 6 Breast 3 Menarche 14
Tanner

Height Velocity (cm/y)

TRC
Breast 2
Curve Magnitude

closed 12
40
eCAP +18
eCAP +24 10
Risser >0
30 Risser 4
Distal radius 8
beginning to
fuse 6
20

4
10
2

0 0
24 18 12 6 0 6 12 18 24 30 36
Time Relative to eCAP

Rapid Moderate Low Height Velocity

Figure 72.1. The basic phases of scoliosis progression during growth along with maturity indicators. The
infantile rapid phase is not shown. (Used with permission from Sanders JO, et al. Predicting scoliosis progression from
skeletal maturity: reliability and validity of a simplified Tanner-Whitehouse classification system in girls with idiopathic
scoliosis. J Bone Joint Surg Am 2008;90:540--553.)

LWBK836_Ch72_p711-720.indd 712 8/17/11 11:14:51 AM

 Chapter 72 Natural History Including Measures of Maturity 713

Figure 72.2. Technique for measuring the ribvertebra angle difference (RVAD) as
described by Mehta. The ribvertebral angle is made from inferiorly between the apical
rib neck and a perpendicular to the apical vertebral inferior end plate. The ribvertebral
angle difference is computed by subtracting the concave from the convex ribvertebral
angle. (Redrawn with permission from Mehta MH. The rib-vertebra angle in the earl diagnosis
between resolving and progressive infantile scoliosis. J Bone Joint Surg 1972;54B:230--243.)

increases and the phase gradually transitions from 1 to 2, the ADOLESCENT

hallmark of progressive curves. Most (83%) resolving curves
have an RVAD less than 20, whereas most (again 83%) pro-
gressive curves have an RVAD of more than 20. This was con-
firmed with even more impressive differences by later authors.
However, double curves present a special problem in that most
of them progress. The RVAD in double curves may be quite
low, but an oblique 11th or 12th convex rib with lumbar rota-
tion is a poor prognostic sign.
JUVENILE
Among juvenile curves, the incidence of progression is 95%,
with only 5% resolving.28 The apical level of the primary curve
is strongly associated with the prognosis as thoracic curves inev-
itably progressed to surgery despite bracing, whereas lumbar
and thoracolumbar curves rarely progressed to the surgical
range. The RVAD and hypokyphosis do not appear to be sig-
nificant prognostic factors. These curves tend to highly pro-
gressive during the adolescent growth spurt.
Convex
Concave
Phase 1
Adolescent curves behave differently on the basis of their curve
patterns and magnitude. In general, lumbar and thoracolum-
bar curves have less progression than do thoracic and double
curves. Thoracic and thoracic predominant double major
curves (Lenke 1 and 3 types) have been found to behave simi-
larly with earlier and more rapid curve progression than other
curve types (Lenke types 2, 4, 5, and 6).29 During the CAP,
rapid progression curves (types 1 and 3) increase an average
1.6 per month, with curve magnitudes eventually exceeding
60. Moderate acceleration curves (all other types) worsen an
average 0.8 per month and reaching 40 to 60. The group
with little acceleration (any curve type) did not reach 40 at
maturity (see Fig. 72.1).
PROGRESSION IN BRACED PATIENTS
The same poor prognostic factors in unbraced patients also
apply to braced patients. Better correction in the brace has a
better prognosis. Whether greater flexibility in untreated curves
carries a better prognosis is unknown. Unfortunately, most
bracing studies use historical controls and the same limited
definition of progression. Only the study by Nachemson and
Peterson22 used concurrent controls, but they defined progres-
sion as more than 5 and use poor maturity determinations.
Overall, their findings indicate that bracing prevents thoracic
and thoracolumbar curves from progressing 6 approximately
40% of the time, compared with untreated patients. Dolan and
Weinstein14 recently published a meta-analysis of bracing and
were unable to find evidence that bracing actually prevents
curves from progressing to surgery.

ASSESSING PHYSICAL MATURITY

Convex Concave
Phase 2
Figure 72.3. The rib phase is distinguished by rib head overlap of
the vertebral body at the curve apex with phase 1 showing no overlap
and phase 2 showing overlap on the anteroposterior radiograph.
(Redrawn with permission from Mehta MH. The rib-vertebra angle in the earl
diagnosis between resolving and progressive infantile scoliosis. J Bone Joint
Surg 1972;54B:230--243.)
Maturation is multidimensional. Because curve behavior is
closely related to maturity, understanding and measuring matu-
rity properly is crucial to those treating scoliosis. Curve progres-
sion is closely connected with the rapid increase in height at the
growth spurt, but measurement of this growth spurt is a prob-
lem in clinical practice. During the first year after birth, the
trunk grows very rapidly, but the rate of growth diminishes dur-
ing each succeeding year until remaining fairly constant from
approximately 5 years until adolescence. There is then a sharp
increase at puberty, known as the adolescent or preadolescent growth

LWBK836_Ch72_p711-720.indd 713 8/17/11 11:14:51 AM

 714 Section VII Idiopathic Scoliosis

3 5
2 4
1

Figure 72.4. The Risser sign. Ossification of the iliac apophysis

usually starts at the anterior superior iliac spine and progresses poste-
riorly. The iliac crest is divided into four quarters and the excursion
or stage of maturity is designated as the amount of quadrant of ossifi-
cation. In the example shown, the excursion is 50% complete and the
Risser sign is 2. On the right, the excursion is complete and the
Figure 72.5. The appearance of phalangeal epiphysis compared
apophysis has fused with the iliac crest, a Risser 5. (Redrawn with per-
with the metaphyseal width. From left to right, the epiphyses are
mission from Lonstein JE. Patient evaluation. In Lonstein JE, Bradford DS,
uncovered, covered (as wide as the metaphysic), capped (edges curl-
Winter RB, Ogilview JW (eds). Moes textbook of scoliosis and other spinal
ing distally), fusing, and fused. The epiphyseal scar may be evident on
deformities, 3rd ed. Philadelphia: WB Saunders, 1995:45--86.)
the fused physis. (Redrawn with permission from Tanner JM, Healey MJR,
Goldstein H, Cameron N. Assessment of skeletal maturity and prediction of
adult height (TW3 method), 3rd ed. London: WB Saunders, 2001.)
spurt. The growth spurt rate is often double or triple the earlier
childhood rate and occurs earlier but to a lesser degree in girls
had only a 4% chance of the curve progressing to a surgical
than in boys. Most of this acceleration is from trunk and spine
range, whereas those with a curve of more than 30 at the time
growth rather than the lower extremities, which have a more
of PHV had an 83% chance of progressing to surgery despite
constant rate of growth and a less conspicuous growth spurt.
bracing. The other study demonstrated that boys behave
Progression of scoliosis of any etiology is strongly associated with
similarly,32 with 100% of curves more than 30 progressing to a
this growth spurt, which usually occurs at ages 10 to 13 for girls
surgical range compared with 14% of lesser curves.
and approximately 2 years later for boys. Classically, maturity has
Unfortunately, the PHV can be identified only retrospectively,
been assessed by chronological age, Tanner staging, menarche,
making PHV a difficult maturity measurement to use clinically.
skeletal age, and the Risser sign.
A number of maturity markers were evaluated and it was
The Risser sign is based on the radiographic excursion of
found that hand skeletal maturity more closely related to the
iliac apophyseal ossification from the anterior superior iliac
CAP and ultimate curve progression than a number of other
spine to the posterior crest (see Fig. 72.4). Unfortunately, Risser
maturity markers, including chronological age, menarchal sta-
1 usually occurs after the growth peak, limiting its use during
tus, and Risser sign.29 The hand skeletal maturity staging was
the important rapid growth phase. Girls are further along their
developed into a simplified and reliable system closely related
growth at any particular Risser stage than boys. To decrease
to curve progression.31
breast radiation, orthopedists generally use posterior to ante-
The epiphyses of the hand form and fuse in an ordered fash-
rior (PA) rather than anterior to posterior (AP) radiographs
ion. The phases of importance are uncovered, covered, capped,
introducing the problem of parallax. This PA Risser sign is
poorly delineated because of the beams angle and varies sig-
nificantly with AP Risser sign.17 Critical reviews of the Risser
sign indicate that it is no better than chronological age and
recommend against its use as the sole maturity indicator.
The pubertal or Tanner stages are highly although not exactly
correlated with the growth spurt and the peak height velocity
(PHV) and are excellent maturity measurements. Girls typically
reach their PHV between stages 2 and 3 for breast development
and stages 2 to 3 for pubic hair development, whereas boys reach 1 2 3 4
theirs between stages 3 and 5 for penile and testicular growth.
Rapid breast development tends to coincide with the accelera-
tion of growth. Unfortunately, pubertal stages have been used
only by a few authors, notably the French, and Tanner staging is
unlikely to be used by most practicing orthopedists.
Menarche is a readily identifiable maturity indicator that
always occurs after the PHV. It is markedly variable compared
with the Risser stage ranging from Risser 1 to Risser 5. Although
5 6 7 8
a number of studies indicate that the risk of progression is sig-
nificantly lower after than before menarche and that it is usu-
Figure 72.6. The various stages of skeletal maturity based on the
ally a reliable sign that growth is decreasing, it is much too vari- hand radiograph. The descriptors of the various stages are described
able for accurate assessments. in Table 72.2. (Used with permission from Sanders JO, et al. Predicting sco-
The adolescent growth peak is best identified by the timing liosis progression from skeletal maturity: reliability and validity of a simplified
of most rapid growth or the PHV. Little et al19 demonstrated Tanner-Whitehouse classification system in girls with idiopathic scoliosis.
that girls with a curve of less than 30 at the time of the PHV J Bone Joint Surg Am 2008;90:540553.)

LWBK836_Ch72_p711-720.indd 714 8/17/11 11:14:52 AM

 Chapter 72 Natural History Including Measures of Maturity 715

TABLE 72.2 Stages of Maturity in Scoliosis

TannerWhitehouse Greulich and Pyle

Stage Key Features Stages Reference Related Maturity Signs
1. Juvenile Slow Digits are not covered Some digits are stage E Female 8  10 Tanner 1
or less Male 12  6
(Note fifth middle
phalanx)
2. Preadolescent slow All digits are covered All digits are stage F Female 10 Tanner 2
Male 13 Starting growth spurt
3. Adolescent rapid The preponderance The digits are stage G Female 11 and 12 Peak height velocity
early of digits are Male 13  6 and 14 Risser 0
capped. Metacarpal Open pelvic triradiate cartilage
2 to 5 epiphyses are (TRC)
wider than their
metaphyses
4. Adolescent rapid Any of distal Distal phalanges stage H Female 13 (digits 2, 3, Girls typically Tanner 3
late phalanges are and 4) Risser 0
beginning to close. Male 15 (digits 4 and 5) Open TRC
Do not make this a
subtle call (refer to
detailed description in
the text)
5. Adolescent All distal phalanges Distal phalanges and Female 136 Risser 0
steadyearly are closed. Others thumb metacarpal Male 15  6 TRC closed
are open stage I Menarche only occasionally starts
Others remain G earlier than this
6. Adolescent Middle or proximal Middle or proximal Female 14 Risser positive (1 or more)
steadylate phalanges are phalanges stages H Male 16 (late)
closing and I
7. Early mature Only distal radius Stage I for all digits Female 15 Risser 4
open Stage G or H for Male 17
May have metacarpal distal radius
physeal scars
8. Mature Distal radius All stage I Female 17 Risser 5
completely closed Male 19

Used with permission from Sanders JO, Khoury JG, Kishan S, et al. Predicting scoliosis progression from skeletal maturity: reliability and
validity of a simplified Tanner-Whitehouse classification system in girls with idiopathic scoliosis. J Bone Joint Surg Am 2008;90:540553.

fusing, and fused (see Fig. 72.5). Covered, in which the epi- comparison with the CAP but will likely produce results similar
physes become as wide as the metaphyses, progresses from the to those of the hand radiograph. Preliminary work indicates
radial to the ulnar side of the hand; capped, in which the that unlike the Risser sign, hand and elbow skeletal maturity
epiphyses curl over the edge of the metaphyses, occurs proxi- provides similar prognostic value for both boys and girls.
mally to distally; and fusion, in which the epiphyses join the
metaphyses, occurs distally to proximally. Radiographically, the
CAP corresponds to epiphyseal capping in the fingers and
metacarpals on the hand skeletal age. This rapid adolescent
curve progression typically lasts approximately 2 to 3 years
beyond the initiation of the CAP when girls are CAP 24. This
also corresponds approximately to Risser 4 and less than 2 cm
of growth remaining. There is approximately a year of slow
growth remaining at CAP 24. Table 72.2 and Figure 72.6
describe the various stages based on the orderly appearance of
the hands growth centers, and Table 72.3 shows the relation-
ship of these stages to curve prognosis.
Charles, DiMeglio, and others from their group in
Montpellier9,12 have recently evaluated skeletal maturity during
adolescence by using the Sauvegrain method (see Fig. 72.7)
and a modification using just the olecranon (see Fig. 72.8) and Figure 72.7. The Sauvegrain scale of elbow maturity. The total
found it highly reflective of the adolescent growth spurt. This score is the sum of each of the component scores. (Used with permis-
latter method is correlated in the figure with the adolescent sion from Dimglio A, et al. Accuracy of the Sauvegrain method in determin-
growth spurt. These elbow methods have not yet been tested in ing skeletal age during puberty. J Bone Joint Surg Am 2005;87:16891696.)

LWBK836_Ch72_p711-720.indd 715 8/17/11 11:14:52 AM

 716 Section VII Idiopathic Scoliosis

The Risk of a Curve Progressing to 50 for a Main Thoracic (Lenke 1) or Double Major
TABLE 72.3
with Larger Thoracic (Lenke 3) Curve Based on Maturity Stage and Curve Magnitude*

Curve/Stage 1 2 3 4 5 6 7 and 8

10 2% (040) 0% (015) 0% (00) 0% (00) 0% (00) 0% (00) 0% (01)

15 23% (469) 11% (158) 0% (02) 0% (00) 0% (00) 0% (00) 0% (07)
20 84% (4098) 92% (5699) 0% (014) 0% (01) 0% (01) 0% (01) 0% (026)
25 99% (68100) 100% (92100) 29% (384) 0% (05) 0% (05) 0% (02) 0% (064)
30 100% (83100) 100% (98100) 100% (47100) 0% (027%) 0% (022) 0% (011) 0% (091)
35 100% (91100) 100% (100100) 100% (89100) 0% (079) 0% (065%) 0% (041) 0% (098)
40 100% (95100) 100% (100100) 100% (98100) 15% (099) 0% (094%) 0% (083) 0% (0100)
45 100% (98100) 100% (100100) 100% (100100) 88% (2100) 1% (099%) 0% (098) 0% (0100)

*The 95% confidence intervals are in parenthesis. The darkly shaded cells are those highly likely to progress to 50, those in white are
unlikely to progress, and the intermittent shading represent those intermediate in progression. (Used with permission from Sanders JO,
Khoury JG, Kishan S, et al. Predicting scoliosis progression from skeletal maturity: reliability and validity of a simplified Tanner-Whitehouse
classification system in girls with idiopathic scoliosis. J Bone Joint Surg Am 2008;90:540553.)

CURVE PROGRESSION AFTER MATURITY
While the most rapid increase in curve size coincides with the
adolescent growth spurt, it is well established that larger curves
continue to increase during adulthood. Three long-term series
are particular impart in this regard.
Ascani et al2 reported on 187 patients followed for between
15 and 47 years after skeletal maturity. Curves less than 40 at
maturity progressed an average of 9 during adulthood. Curves
more than 40 progressed an average of 20. Cardiopulmonary
failure was also more common occurring in 35% in those with
more than 40 and 10% in those with less than 40 at maturity.
They did not note the patients smoking history. Patients
appearance generally improved with age.
Edgar16 reported on 78 patients followed between 10 to 27
years after maturity with a mean age of 33.7 years at follow up
and found continued progression in adulthood of 0.5 to 1.0
per year. The largest increases were in thoracic curves 90 to
100 at maturity. Lumbar curves had less increase in Cobb
angles but more rotational changes and lateral subluxation,
particularly at the midlumbar level. The thoracic component of
double curves was the most benign.
The longest and the most detailed follow-up studies are
from the University of Iowa.10,23,3437,39,40 The results of its study
concerning back pain and pulmonary status were discussed
previously. Its recently reported 50-year follow-up included
patients lost in their prior reports, resulting in a 93% follow-up
of 219 patients. Curves less than 30 had little tendency to prog-
ress in adulthood. The maximum progression occurred in tho-
racic curves 50 to 75, with progression 0.75 to 1.0 per year.
The progression continued at 50-year follow-up. Additional risk
factors for progression are listed in the Table 72.4. The fifth
lumbar vertebra located above the pelvic intercrestal line in
lumbar curves and translatory shifts or subluxation of the
midlumbar spine in lumbar and thoracolumbar curves were
also risk factors for progression. Double curves had a tendency
for the lumbar component to increase more than the thoracic
curve and balance the thoracic curve.

Risser 0 Elbow
fusion

Menarche

Risser 1
Risser 2
Risser 3
Y-cartilage Risser 4
closure Risser 5

Girls 11 11.5 12 12.5 13 13.5 14 14.5 15 15.5 years

Boys 13 13.5 14 14.5 15 15.5 16 16.5 17 17.5 years

5
4.5
Growth velocity

4
3.5
cm/6 mo

3 Figure 72.8. Olecranon maturity as a simpli-

2.5 fied Sauvegrain scale relative to the adolescent
2
1.5 growth spurt. (Used with permission from Charles YP,
1 et al. Skeletal age assessment from the olecranon for
0.5
0 idiopathic scoliosis at Risser grade 0. J Bone Joint Surg
Lower limbs Sitting height Am 2007;89:27372744.)

LWBK836_Ch72_p711-720.indd 716 8/17/11 11:14:52 AM

 Chapter 72 Natural History Including Measures of Maturity 717

Factors Related to Progression in Curves More

TABLE 72.4
Than 30 at Maturity

Thoracic Lumbar Thoracolumbar Combined

Cobb angle 50 Cobb angle 30 Cobb angle 30 Cobb angle 50
AVR 30 AVR 30
AVR 30 Curve direction Translatory shift
Ribvertebra angle L5 to intercrestal line
difference 30 Translatory shift

WHY CURVES PROGRESS approximately age 8, followed by volumetric growth11,27 (see

The most commonly accepted mechanism in the immature is
the initial development of a small curve then progressing
when the growth cartilage on the concave side becomes over-
loaded and its growth inhibited. More recent work indicates
that initial scoliosis progression during the growth spurt occurs
through the intervertebral discs with bone deformity occurring
later implying an initial soft tissue imbalance with the Heuter
Volkmann principle acting later in maturity. It is thought that
adult deformities progress primarily through disc degenera-
tion, but this has not been studied in detail.
CONSEQUENCES OF SCOLIOSIS
Influence of Spinal Deformity on the Lungs
Large curves can result in significant restrictive lung disease
and cor pulmonale. Unfortunately, there are only a few
high-quality studies evaluating chest and lung development
and long-term pulmonary prognosis in scoliosis. Lung develop-
ment occurs with substantial alveolar number increase until
Fig. 72.9). Ultimately, it is probably the surface area of ventilated
and perfused alveoli in relationship to oxygen demand that mat-
ters. The earlier the onset and larger the curve, the more likely
is pulmonary failure. Juvenile and particularly infantile curves
are much more likely to result in early to mid adult life pulmo-
nary restriction than adolescent curves, which rarely cause prob-
lems except in the presence of marked thoracic lordosis. A
Scandinavian 20-year follow-up study24 found that respiratory
failure occurred only in patients with a low vital capacity at ear-
lier follow-up who also had curves greater than 110. Because
these patients, like all adults, further decreased their vital capac-
ity through the normal aging process, they subsequently decom-
pensated. Weinstein et al,40 in a long-term follow-up of untreated
adolescent patients, found pulmonary function failure only in
those with thoracic curves more than 100 to 120, except in
smokers who fared worse. Double curves did not demonstrate
pulmonary problems although their curves were not as severe.
Overall, in untreated scoliosis, only infantile and juvenile
curves or severely lordotic thoracic curves of high degree are
likely to result in pulmonary problems, but typical adolescent

600

500
100%
50%
30% (Dimeglio)2
Total Number of Alveoli (x106)

400 6.7%

300

200

100

0
Figure 72.9. Growth of the alveoli relative to age and 0 2 4 6 8 10 12 14 16 18 20 Age (yr)
chest volume. (Used with permission from Campbell RM, Smith Dunnill11 Weibe51 Angus Davies Hieronymi54,55
MD. Thoracic insufficiency syndrome and exotic scoliosis. J Bone and and Reid53
Joint Surg 2007;89:108122.) Thurlbeck52

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 718 Section VII Idiopathic Scoliosis
curves do not markedly affect long-term pulmonary function
despite some restrictive disease.
THORACIC INSUFFICIENCY SYNDROME
Campbell has popularized the concept of thoracic insufficiency
syndrome (TIS).6,7 According to this concept, the chest is a ven-
tilatory box of which the spine forms one portion but which
contributes substantially to the longitudinal growth of the box.
Early spine fusion or insufficient spinal growth such as in mul-
tiple congenital vertebral anomalies will cause a decreased axial
growth of the box with subsequent decreased capacity. In addi-
tion to TIS from volume depletion, pulmonary function can be
decreased by poor mechanical action from weak muscles, fused
ribs, or inefficient costal motion, or by intrinsic lung disease.
MORTALITY
Early studies indicating an extremely high mortality rate for
idiopathic scoliosis suffered from significant methodological
problems including lack of radiographs and imprecise diagno-
sis. Two long-term studies have evaluated mortality of idiopathic
scoliosis.25,38 As mentioned earlier, Pehrsson et al25 found sig-
nificantly increased mortality for both infantile- and juvenile-
onset scoliosis but not for adolescent scoliosis compared with
the general population. Infantile-onset scoliosis had the high-
est mortality rate, whereas adolescent idiopathic scoliosis even
of severe degree did not have increased mortality.
BACK PAIN
Biomechanical concepts of back pain predict a high incidence
of degenerative arthritis and back pain in patients with scolio-
sis. This has only been partially confirmed. With back pain
being highly prevalent, many studies are flawed by selection
bias (e.g., only including patients seen in a low back pain
clinic), lack of controls, and the inability to distinguish idio-
pathic curves from adult de novo curves.
Back pain is reported in approximately 32% of adolescents
with idiopathic scoliosis,26 with an increased incidence toward
maturity not associated with the degree of curvature.
Unfortunately, because back pain is highly prevalent in adoles-
cents and increases with age, this information is difficult to
interpret. Studies comparing adults with scoliosis with age-
matched controls frequently suffer selection bias. Two longitu-
dinal studies10,20,23,34,35,37,39,40 examined back pain over time.
The Iowa study compared age-matched controls to scoliosis
patients followed since adolescence. At 50-year average fol-
low-up, the untreated scoliotic patients had a back pain inci-
dence of 77% versus 37% of controls, which did not vary with
the type of curve,37 and 61% of scoliotic patients had chronic
pain compared with 35% of controls. It was generally unrelated
to radiographic changes except in the presence of lumbar and
thoracolumbar translatory shifts. Despite the discomfort, scoli-
osis did not affect patients job status, and they remained quite
high functioning and with no differences from controls in their
ability to work and perform everyday activities. In the Ste-Justine
study of adolescent idiopathic scoliosis,20 follow-up was much
shorter, with a median of 14 years, but the study had markedly
similar results with current pain in 44% of patients compared
with 24% of controls and pain within a year in 73% of scoliosis
LWBK836_Ch72_p711-720.indd 718
patients compared with 56% of controls. The scoliosis patients
also reported more intense pain, but this did not vary signifi-
cantly with the type of curve or magnitude. Patients with severe
curves (defined as 40) and those surgically treated had more
difficulty with lifting, walking, pain control, and in frequently
having to lie down during the day because of pain.
These studies indicate a higher incidence and severity of
back pain in scoliosis patients than in controls but with little
physical disability compared with controls.
PREGNANCY
Some early works demonstrate a possible deleterious effect of
pregnancy on scoliosis.3,5 A subsequent more extensive review
comparing scoliosis patients who had at least one pregnancy
with those who had never been pregnant4 found no increase in
curve magnitude, back pain, or obstetrical outcome in scoliosis
patients. There appears to be no difference in deliveries
between patients with and without scoliosis.33
Appearance: The spines appearance is often a major con-
cern and a frequently unspoken but important treatment out-
come. The term cosmesis for disfigurement from a spinal
deformity is not appropriate. Cosmetic, derived from the
Greek word kosmetikos, meaning skilled in adornment, is not
generally appropriate when applied to deforming conditions,
which are more accurately described by appearance and dis-
figurement. The measurement of scoliosis appearance is now
possible,30 but our understanding of its importance remains in
its infancy. The appearance portion of the SRS instrument is its
most responsive domain to surgical treatment.30 The Spinal
Appearance Questionnaire (SAQ) (Fig. 72.10) has recently
been developed, which addresses several domains of spinal
deformity shape, and its testing is promising for reliability and
validity.
Function: Very few scoliosis studies have evaluated the long-
term functional outcomes of either untreated or treated scolio-
sis by using validated health-related quality-of-life instruments.
These instruments were not available until the last decade, but
their use will be essential in future studies both as disease-
specific instruments for various aspects of spinal deformity and
as general health instrument comparing spinal deformity
patients with patients with other disorders.
SUMMARY
The factors well known to predict curve progression are lesser
maturity and larger curve magnitude. Recent findings have
delineated the important role of skeletal maturity as the most
important physiological measure of maturity for scoliosis. There
is a strong tendency toward progression of larger curves in
adulthood although the patients function is not generally infe-
rior to that of controls.
EPILOGUE
In light of the literature findings that scoliosis is fatal only in
infantile and juvenile or congenital curves and except for very
large curves, adolescent curves result in little cardiopulmonary
disability or back difficulties, the goals of treatment become
cloudy. Ultimately, the question of why we should treat scoliosis
assumes importance. If scoliosis treatments were inexpensive
8/17/11 11:14:52 AM
 Chapter 72 Natural History Including Measures of Maturity 719

Figure 72.10. The Spinal Appear-

ance Questionnaire. (Used with permis-
sion from Sanders, et al. The spinal
appearance questionnaire: results of reliabil-
ity, validity, and responsiveness testing in
patients with idiopathic scoliosis. Spine
2007;32(24):27192722.)

LWBK836_Ch72_p711-720.indd 719 8/17/11 11:14:53 AM

 720 Section VII Idiopathic Scoliosis
with perfect results and no risks, it is doubtful anyone would
object to treating even minor curves. With current knowledge
and techniques, we are far from this goal and treatment for
scoliosis must balance risks, costs, and perceived benefits. The
ultimate decisions on treatment methods and the balancing of
these three factors will likely vary by culture and family, patient
personality, and the ability or desire to pay for the treatment.
Almost all of the literature looking at the consequences of sco-
liosis is from Western cultures, and the generalizability to other
cultures is unknown.
For early-onset curves, the goal must clearly be to afford suf-
ficient and directed spine growth for good adult pulmonary
function. For large thoracic curves, the goal of improving chest
wall mechanics, lung function, and quality of life are also gener-
ally clear. For most adolescent curves, the answers take a differ-
ent direction. Scoliosis surgery for minor appearance issues can
cross over into cosmetic surgery similar to breast augmentation
for normal but small breasts or antidepressants for mild mood
disturbances. From this viewpoint, increasingly risky methods of
minor appearance improvement without substantial additional
benefits should be viewed skeptically. Scoliosis is, in its more
marked forms, a severe deformity. There is something intrinsi-
cally humane about wanting to be normal as other humans as
demonstrated in other disorders, such as the child with a cleft lip,
the woman with a chest disfigurement from breast cancer resec-
tion, or a person with a traumatic or congenital amputation. No
one disputes the importance of attempting to restore these peo-
ple to wholeness, even if they are able to function as well as any-
one else without treatment. The disfigurement from scoliosis is
similar. The importance of being whole and similar to others is a
part of being human. But, like these other disorders, treatment
involves risk and cost and requires balancing these factors.
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