THE MANAGEMENT OF INFANTS WITH SCOLIOSIS

J. I. P. JAMES, EDINBURGH, SCOTLAND

From the University Department of Orthopaedic Surgery, Edinburgh

This is a study of children who first attended as infants with either progressive infantile idiopathic
scoliosis or congenital scoliosis. All had a pattern of scoliosis in which early and damaging deterioration is
inevitable. The infants were treated from before the age ofthree, initially by plaster casts and then a Milwaukee
brace, followed at about the age of ten by correction and fusion. The cases were then observed to the end of
growth or near that point. In the main study there were twelve cases, six of progressive infantile idiopathic
scoiosis and six of congenital scoliosis, which were followed through this long period. Only one of the twelve
had a curve worse at the end of growth compared with the initial radiograph as an infant; this one curve
had increased only 16 degrees in almost as many years. Although small, the series does show that it is nearly
always possible to control even the most serious scoliosis in an infant, if it is tackled early and unremittingly.
There are supportive studies ofchildren who have partially completed this regime, and interim results in a newer
group of children with spina bifida and scoliosis.

Scoliosis still presents some of our most difficult methods, which include observation, hinging plaster
problems, problems that are indeed at times insoluble. jackets, the Milwaukee brace and spinal fusion at the
This is particularly true of infants under three years of age of ten or eleven. The number in whom an end-result
age with progressive scoliosis, for over the next fifteen is available is relatively small because of the many years
to eighteen years of growth their curves are likely to required for a continuous study from infancy to skeletal
progress relentlessly and may lead to an uncomfortable maturation.
death from cor pulmonale in early adult life. A severe While scoliosis in the older child may result from
scoliosis causes compression of the lungs with consequent any one of fifty different diseases, in the infant there are
difficulty in aeration of the alveoli, and right heart hyper- only a few causes, with a great predominance of the
trophy because of the difficulty in circulating blood infantile idiopathic and congenital varieties. A few
through the lungs. There is then a gradual failure of infants have been seen with scoliosis from Morquio’s
both respiratory and cardiac function-cor pulmonale disease and
neurofibromatosis, but they are too few to
(Zorab 1973). Curves over l0degrees ifidiopathic, or less consider. In congenital scoliosis a significant degree of
if congenital or paralytic, are sufficient to cause cor curvature is frequently delayed until early adolescence;
pulmonale. In Sweden 30 per cent of such patients are infants with a visible congenital scoliosis before the age
granted a disability pension (Bjure and Nachemson 1973), of three are uncommon and the curvature usually be-
and the mortality is twice that expected of their age comes very severe ; this small group with a high risk of
group. Disability and death are almost invariably cardiac early deterioration concerns us here.
and respiratory in origin. Few months go by in which
one of these tragic late cases is not referred to us, a
INFANTILE IDIOPATHIC SCOLIOSIS
reminder, if one were needed, of the penalties of doing
nothing. On the other hand successful management is This disorder affects the majority of infants with scoliosis,
not easy. The parents and the child must face together and indeed its frequency in Great Britain as compared
many wearisome years of visits to clinics, the wearing of a with North America is quite astonishing (Wynne-Davies
clumsy cast or brace, and finally spinal fusion. 1975). A study of 200 cases of idiopathic scoliosis seen
Many hundreds of older children whose curves between 1968 and 1972 indicates the proportion of cases
started in infancy have been seen at the scoliosis clinic of early onset (Table I).
held initially in London from 1948 to 1958 and subse-
quently in Edinburgh. The great majority came too late, TABLE I
and it was they who taught us how serious is the result
Two HUNDRED CONSECUTIVE CASES OF IDIOPATHIC SCOLIOSIS
of neglect. It has therefore seemed worth while to review
those cases from the Edinburgh clinic who came before Infantile (onset before 3 years) . . . . 82
the age of three and who have remained under care
Juvenile (onset from 4 to 9 years) . . . 34
throughout growth, amply long enough to demonstrate
the results that can be achieved by attempts to prevent Adolescent (from 10 years to end of growth) . 84
serious curvature. In our view spinal fusion in the very 200
young is wrong, so this assessment is of our present

Professor J. 1. P. James, M.S., F.R.C.S., University Department of Orthopaedic Surgery, Clinical Research Unit, Princess Margaret Rose
Orthopaedic Hospital, Fairmilehead, Edinburgh EHIO 7ED, Scotland.

422 THE JOURNAL OF BONE AND JOINT SURGERY

 THE MANAGEMENT OF INFANTS WITH SCOLIOSIS 423

In the present series there was a total of 120 cases the double structural curve scoliosis of infants, before it could
of infantile idiopathic scoliosis to be considered. Plagio- be suspected clinically. These are a very oblique eleventh or
twelfth rib on the concave side and a rib-vertebra angle now
cephaly is present at an early age in all infants with greater on the convexity (Fig. 4). In this series double curves
idiopathic scoliosis ; the typical facial and cranial de- in two infants have undergone resolution, an occurrence not
formity is shown in Figure 3. As is now well known,
the majority show spontaneous resolution of the TABLE II
structural curve and have so-called resolving infantile INFANTILE IDIOPATHIC RESOLVING SCOLIOSIS
scoliosis. In contrast the minority have progressive YEAR OF RESOLUTION OF CURVATURE IN EIGHTY-SEWN CASES

curves, and if not treated deteriorate steadily, forming a

group comprising some of the most badly deformed Firstyear . . . . . . I6cases
children we see.
Second year . . . . . . 26 cases

Third year . . . . . . 23 cases

Resolving infantile idiopathic scoliosis
In the last sixteen years ninety cases have been followed Fourth year . . . . . . II cases
through to resolution. In three cases there is no record
Fifth year . . . . . . 6 cases
of when this happened, but in the other eighty-seven the
curves had all disappeared by the sixth year (Table H). Sixth year . . . . . . 5 cases

TABLE III
PROGRESSIVE INFANTILE IDIOPATHIC SCOLIOSIS
TREATMENT BY PLASTER, Baca AND FUSION

Case Initialcurve After plaster After brace After fusion

number (degrees) (degrees) (degrees) (degrees)

I 48 6 25 25

2 54 39 65 70

3 61 27 40 39

4 70 38 60 52

5 78 57 67 55

6 86 55 83 44

TABLE IV
THE RESULTS OF PLsmR JACKETS AND BRACE IN
Concave
PROGRESSIVE INFANTILE IDIOPATHIC ScOLIOSIS
side
FIG. I
To show the method ofmeasuring the rib-vertebra angles at Case Initial curve After plaster In brace
theapex ofthecurve(Mehta 1972). In this tracing ofa radio- number (degrees) (degrees) (degrees)
graph the rib on theconcaveside forms thegreater angle with
the vertebra. The nb-vertebra angle difference (R.V.A.D.) is 1 42 50 22
therefore positive for theconcaveside. Because thedifference
here is 38 degrees this isa progressivecurve. Notice also that 2 73 - 55
the head ofthe rib is partly behind the apicalvertebra on the
convexity, another indication of a progressive curve. 3 73 21 45

4 38 22 24
There was no other feature of interest to report, but one
must stress the importance of Mehta’s (1972) observation 5 53 - 17

that the difference in obliquity of the ribs on either side 6 42 10 67

of the apical vertebra is of great value in the differential
7 73 36 20
diagnosis between resolving and progressive scoliosis
(Fig. 1). When the rib-vertebra angle difference (RVAD) 8 30’ 7 2’

is less than 20 degrees, resolution will occur (Fig. 2); a

difference of more than 20 degrees usually indicates a I R.V.A.D. 30 degrees. ‘ Out of brace since 1968.

progressive curve. The rib-vertebra angle is greater on

the concavity in single curve scoliosis because the rib on previously reported. There were four other infants with
double curves;
one has not increased during ten years and
the concave side is less oblique to the vertebra.
the other three, as expected, behaved well but are now in
Double structural curves-Mehta described two radiological Milwaukee braces and progressing favourably at the ages of
features indicating the presence of a second structural curve, eleven, eleven and eight years respectively.

VOL. 57-0, No. 4, NOVEMBER 1975

424 J. I. P. JAMIS

1O#{149}68 .J.j
FIG. 2
Radiographs ofa child who had a resolving scoliosis. The R.V.A.D. was 16 degrees. Note that all the rib heads on the convexity
were clearly visible, a valuable sign of a resolving scoliosis. Eighteen months later the curve had disappeared.

FIG. 3 FIG. 4
A photograph showing plagiocephaly, a characteristic facial A case of double structural idiopathic scoliosis. In such cases
deformity associated with infantile idiopathic scoliosis, both the rib-vertebra angle is greater on the convexity than on the
resolving and progressive. Seen from above there is hypo- concavity ; the difference, usually small, is written as a negative
plasia of the right cheek and brow. The child would therefore figure to indicate this. Note the obliquity of the eleventh and
have a curve convex to the right. Note that the left ear is twelfth ribs on the concave side of the thoracic curve, another
turned forward, a characteristic feature of plagiocephaly. strong indication of a double curve.

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 THE MANAGEMENT OF INFANTS WITH SCOLIOSIS 425

without benefit of effective treatment, and there is much

previous. information from which to gauge the severe
prognosis (James 1954; Scott and Morgan 1955). Figure 5
shows a typical case. Figure 6 conveys the expected
outcome of progressive curves left untreated. This histo-
gram and the papers just referred to indicate very clearly
the poor prognosis of untreated progressive infantile
idiopathic scoliosis : by the age of five years 57 per cent
have already passed the point of no return, that is a
70 degree curve, with the inevitable cor pulmonale and
other irreversible problems.
If an infant is seen with even a very small curve but
with a rib-vertebra angle difference exceeding 20 degrees,
one must consider this to be progressive. Treatment
begins with a hinging plaster jacket until the child is
old enough to wear a Milwaukee brace at two to three
years of age. A child first seen at two and a half to three
years goes straight into a brace. The brace is worn until
ten or eleven years, when correction and fusion are
usually performed.
There were six children in this series who came before
the age of three, had a full attempt at control and were
followed to fusion and after, over total periods of more
- .3. 5
than ten years. The effects on the curvatures after each
A typical case of neglected progressive infantile idiopathic scoliosis
in a boy of I 3 who still has several years to grow and who will stage of treatment are recorded in Table III. Only in
become worse. He will almost certainly die of cor pulmonale Case 2 was the final curve after fusion, 70 degrees, greater
between 30 and 40.
than that recorded in the first radiograph, 54 degrees,
some ten years before. The other five children had
smaller measurements after fusion than as infants, which
Progressive infantile idiopathic scoliosis
clearly demonstrates that such curves can usually be
In this discussion the progressive curves are of much
controlled. Figure 7 illustrates a typical sequence.
greater interest, for we are concerned with evaluating
Another eight infants treated by plaster and brace,
our ability to prevent serious deformity in its earliest
or by brace alone, have not reached the age for fusion.
stage. Many such patients have of course been seen late
Seven of the curves have stabilised in the brace and from
experience are now unlikely to deteriorate (Fig. 8). One
25 curve is worse (Case 6). The details are recorded in
23 Table IV. The first seven children will be maintained in
SrrnO
20 a brace until old enough for fusion, when their curves
may be expected to be still further reduced by the
15 14
corrective procedures undertaken immediately before that
12 operation. Case 8 clearly does not require fusion, though
)O all the evidence suggests that this was a progressive curve.
9
“Pseudo-cures”

Agegroups

Numberof
chi’dren
: 0-5
47
ii
6-10
37
J
2

h-maturity
23
There

extent
tion
were
exceptionally
of a cure.
is available,
well
ten patients
in plaster
Now that Mehta’s
and with
and

hindsight,
brace,
method
who

these
some
appeared
to the
of classifica-
ten would
to have done

seem to have had resolving curves. The curves were either

FIG. 6
rather severe and therefore seemed of the progressive
A histogram which shows the poor prognosis of progressive
infantile idiopathic thoracic scoliosis. The maximum angle type, or more commonly the reason for treatment was a
of curvature in these three age groups of children is from marked increase in the curve.
the radiograph taken on referral and before any effective
treatment. Of forty-seven children under 5, four already
had curves over 100 degrees and another twenty-three had
curves over 70 degrees; thus 56 per cent already had an CONGENITAL SCOLIOSIS
incurable curve which would cause great disability and
shorten their lives. In the middle group of thirty-seven, the Obviously the vertebral anomalies which cause congenital
larger number had curves over 100 degrees. In the third scoliosis are present in infancy. However, most of the
group oftwenty-three coming to us late, only two had curves
less than 70 degrees. affected infants do not present with curvature in the

VOL 57-B, No. 4, NOVEMBER 1975

4
426 J. 1. P. JAMES

FIG. 7
Case 3-Infantile idiopathic thoracic scoliosis measuring 61 degrees and with an R.V.A.D. of 45 degrees. The curvature being clearly
progressive, the child was put into a hinging plaster jacket and the curve was reduced to 27 degrees eighteen months later. Six years later
still, and just before fusion, the curve measured 40 degrees in the Milwaukee brace. Two years after correction and fusion it was 39 degrees.

t.j. 8
This child came rather late with a curve of 73 degrees and an R.V.A.D. of 71 degrees. The photograph shows the amount of rotation when
he first presented; this will persist and remain very ugly and is a major reason for early treatment. A brace was immediately applied
because he was old enough for this. As expected, the correction to 55 degrees is poor but will be maintained at about this level until fusion.

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 THE MANAGEMENT OF INFANTS WITH SCOLIOSIS 427

first three years because the irregular growth has not had reached the end of growth (Fig. 9). One case needed
time to deform the spine. Indeed, congenital anomalies fusion in adolescence because of late deterioration.
which cause a visible curve of the spine in the first three Only six patients have had treatment from infancy
years usually have an exceptionally poor prognosis, which to near skeletal maturation, but the results are very
is very evident from the terrible problems seen in Un- instructive (Table V). The prognosis in congenital scoli-

FIG. 9
A case of congenital scoliosis. In 1965 this child had a curve
measuring 20 degrees, but seven years later, without treatment, it
had reduced to 8 degrees.

FIG. 10
A case of very severe congenital scoliosis, 97 degrees when first seen in 1959, and 1 1 1 degrees two years later when the child was old
enough for a brace. Some six years later still, the curve, then reduced to 100 degrees, was corrected and fused, with little change as
expected. Two years after fusion it measured 95 degrees, only 2 degrees less than some thirteen years earlier but much better than if
nothing had been done.

treated cases and which has been very well documented osis is not so well defined as in other varieties, but one
recently (Winter, Moe and Eilers 1968). In this series would have expected all of these six curvatures to get
there were forty-nine such cases to be considered. Nine- much worse, and none did so (Fig. 10).
teen required no treatment during the period of observa- As in the idiopathic group, a number of children
tion, which was usually many years and in some cases are under treatment by brace and are apparently stabilised

VOL. 57-B, No. 4, NOVEMBER 1975

428 J. I. P. JAMES

13365
FIG. 11
A severe congenital curve with bony fusion on the concavity
indicating a poor prognosis. Nevertheless the curve was reduced
from 74 to 45 degrees in the brace and held at that for some years.
Fusion is to be performed quite soon.

/.
.7
...i. .

-I ..

-- .

-7. 12

A case of high thoracic congenital scoliosis. The first radiograph shows the typical congenital deformities and a curve of
24 degrees, which increased to 53 degrees six years later. The child came to us at this stage showing a very ugly deformity
with elevation of the shoulder-neck line from the vertebral rotation.

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 THE MANAGEMENT OF INFANTS WITH SCOLIOSIS 429

but not yet old enough for fusion. Most of them have osis. Those with paraplegia often develop severe curves,
been in a brace for five years or more. AlIoI the eleven whereasothers may have severe but flexible curves. There
such cases have improved (Fig. 11). were thirteen children with a major spina bifida and
The very ugly high thoracic curve is :aspecii
case; scoliosisuncler long enough study to be considered here.
it must never be allowed to increase over 30 degrees. Six have progressed slowly and are receiving no treatment;
The child shown in Figure 12 came to us late and has seven are wearing a Milwaukee brace. This brace presents
the typical and irreversible raised neck-shoulder line from two difficult problems-pressure sores may develop over
rotation of the vertebrae. anaesthetic skin, and the wearing of both a brace and
Kyphoscoliosis with its risk of paraplegia is a special calipers effectively stops walking. A compromise under
and very important problem which it is not proposed to trial is to use the brace at night and calipers during the
consider here. day. At present we seem to be as successful as in the
TABLE V other groups, but the difficulties with the brace may well
THE RESULTS OF TREATMENT OF CONGENITAL SCOLIOSIS BY PLASTER, defeat our aims.
BRACE, THEN FUSION

Case Initial curve After plaster After brace After fusion

number (degrees)
COMMENT
(degrees) (degrees) (degrees)

I
Despite the arduous demands on all concerned, there is
76 59 62 60
no doubt that one can hold both progressive infantile
2 III - 83 95
idiopathic and congenital curves from infancy onwards,
3 61 38 46 30 and that in the few that do show an increase (in this
series only two) the increase is very much less than in
4 70 58 50 60
untreated cases. These excellent results must encourage
5 47 - 47 25 all of us to act more vigorously. The alternative may
6 40 - 30 25 be disaster.
The series reported here is small for obvious reasons,
but it has been remarkably and unexpectedly successful.
SPINA BIFIDA WITH SCOLIOSIS
Exceptional cases of failure are bound to occur in both
In this final group, children with primarily a spina bifida the idiopathic and the congenital groups, and then earlier
problem are beginning to present with developing scoli- correction and fusion may have to be performed.

I am grateful to many orthopaedic colleagues who continue to refer these cases, and to Miss R. Wynne-Davies who compiled the figures
given in Tables I and II.

REFERENCES
Bjure, J., and Nachemson, A. (1973) Clinical Orthopaedics
Non-treated and Related
scoliosis. Research, 93, 44-52.
James, J. I. P. (1954) Idiopathic scoliosis. Journal ofBone andJoint Surgery, 36-B, 36-49.
James, J. I. P. (1967) Scoliosis. Edinburgh and London : E. & S. Livingstone Ltd.
James, J. I. P., Lloyd-Roberts, G. C., and Pilcher, M. F. (1959) Infantile structural scoliosis. JoiirnalofBoneandJoint Surgery, 41-B, 719-735.
Mebta, M. H. (1972) The rib-vertebra angle in the early diagnosis between resolving and progressive infantile scoliosis. Journal of Bone
andJoint Surgery, 54-B, 230-243.
Scott, J. C., and Morgan, T. H. (1955) The natural history and prognosis of infantile idiopathic scoliosis. Journal ofBone andJoint Surgery,
37-B, 400-413.
Winter, R. B., Moe, J. H., and Eilers, V. E. (1968) Congenital scoliosis. A study of 234 patients treated and untreated. Journal of Bone
and Joint Surgery, 50-A, 1-47.
Wynne-Davies, R. (1975) Infantile idiopathic scoliosis. Journal ofBone andJoint Surgery, 57-B, 138-141.
Zorab, P. A.(I967)The medical aspects ofscoliosis. In Scoliosis by J. I. P. James. 238-246. Edinburgh and London : E. & S. Livingstone Ltd.

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