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Experimental and Molecular Pathology 74 (2003) 326 –335 www.elsevier.com/locate/yexmp

Regression of juvenile idiopathic scoliosis


William E. Stehbens* and Rachel L. Cooper
Department of Pathology and Molecular Medicine, Wellington School of Medicine and Health Sciences, Wellington, New Zealand

Received 21 August 2002

Abstract
For a young scoliotic boy the customary “wait and watch” management program for rapidly progressive juvenile idiopathic scoliosis was
considered unsatisfactory in view of the poor prognosis. The management program devised was based on the congenital postural induction
concept of scoliosis with progression accruing from mechanically induced bioengineering fatigue, cumulative molecular scissions, laxity of
ligaments, and secondary bone deformation. A coexisting pelvic tilt with restricted movement of the hip and shoulder joints was overlooked
initially. Possibly induced simultaneously with the scoliosis, it is considered a contributory factor in scoliosis progression and requires early
diagnosis and correction. The rapid improvement in this child’s spinal status achieved by physiological traction and specifically designed
exercises was such that as a preventive measure the technique warrants further clinical assessment on young scoliotics.
© 2003 Elsevier Science (USA). All rights reserved.

Keywords: Idiopathic scoliosis; Congenital postural scoliosis; Conservative exercise program; Vertebral traction; Scoliosis regression

Introduction clinically manifest from 3 to 10 years of age. This arbitrary


classification does not preclude the possibility that juvenile
Despite a poor prognosis, juveniles with early idiopathic scoliosis commences earlier or even in utero. The need to
scoliosis currently receive only periodic surveillance in the separate juvenile from infantile scoliosis has been ques-
hope that the spinal curvature will spontaneously regress, tioned (James, 1965; Mehta, 1992; Stehbens, 2002) as the
there being no effective nonoperative therapy available to former seems to accrue from undetected curvatures in in-
reverse or stabilize the deformity. This analysis of the man- fancy (Dickson, 1985).
agement is aimed at improving the prognosis and hopefully The little known congenital postural scoliosis (Browne,
at preventing the development and progression of this tragic 1956, 1965; Dunn, 1976a, 1976b) has been attributed to
deformity. compression deformity in utero as a result of rapid fetal
growth in late pregnancy, diminishing amniotic fluid, and
Scoliosis and posture impaired mobility, the latter indicated by diminished kick-
ing and fetal inability to change position (Dunn, 1976a).
In approximately 15–20% of cases of scoliosis the initial
While mobile the body appears to grow normally in utero.
cause is known and in the remainder the so-called idiopathic
When comparatively immobile in a cramped and curved
scoliosis is likely to be postural in nature (Stehbens, 2002).
position, the relatively fixed spine and ligaments maintain
Like hallux valgus, scoliosis should be regarded as a bio-
growth in this incipient scoliotic position with muscles
mechanical deformity highly prone to further progression.
By convention, idiopathic scoliosis is of the infantile type probably underdeveloped rather than atrophic from little
when detected before 3 years of age and juvenile when use. After birth, when unrecognized and uncorrected, the
small curvature is perpetuated as a “position of comfort”
and ever so slowly and insidiously progresses to severe
* Corresponding author. Department of Pathology and Molecular Med-
icine, Wellington School of Medicine and Health Sciences, P.O. Box 7343,
scoliosis especially as the upright position is assumed. Most
Wellington South, New Zealand. Fax: ⫹64-4-389-5725. of these curves are said to undergo spontaneous resolution
E-mail address: wstehbens@wnmeds.ac.nz (W.E. Stehbens). (Dunn, 1976a; James, 1954a) with an undefined number

0014-4800/03/$ – see front matter © 2003 Elsevier Science (USA). All rights reserved.
doi:10.1016/S0014-4800(02)00014-X
W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335 327

passing unnoticed and presenting later as juvenile or ado- with no propensity to progression. Previously Dickson et al.
lescent scoliosis. The current practice of ignoring curves (1980) had attributed the pelvic tilt to unequal leg length, a
with Cobb angles under 10° masks an uncertain number of concomitant of the spinal curvature or pelvic asymmetry per
more slowly growing deformities (Stehbens, 2002). Other se whereas the latter is more likely to be secondary to severe
idiopathic scoliotics may develop habitual, undesirable pos- scoliosis or to develop simultaneously under the same pres-
tures in which the spine becomes relatively fixed due to sure. They also postulated that a substantial proportion of
growth of spinal ligaments in a scoliotic posture with later scolioses is entirely or partially secondary to the sacral tilt,
progression during a growth spurt or ligament failure. that scoliosis did not progress in children with curves solely
Congenital postural deformity initially defined by due to a sacral tilt, and that compensatory lumbar scoliosis
Browne (1936, 1956) may be more important in idiopathic (commonly produced by a sacral tilt secondarily to inequal-
scoliosis than currently believed. Infantile “idiopathic” sco- ity of leg length) is never progressive.
liosis has been associated with plagiocephaly and dysplasia The pelvic tilt in scoliotics is deserving of more atten-
of the hip (Browne, 1965; Dunn, 1976b; Lloyd-Roberts and tion. It is greatest in small curves and mostly associated with
Pilcher, 1965; Wynne-Davies, 1968, 1975) which like hip lumbar and thoracolumbar curvatures (Dickson, 1983). Re-
dislocation, bat ears, and torticollis can be produced by fetal garding it as inconsequential is unwarranted for whatever
compression in utero, the posture perpetuated postnatally as the cause, the result is spinal instability unless the L5 ver-
a position of comfort. With the femoral head held in its tebra is wedge shaped to compensate. Its frequent associa-
proper position, hip joints develop normally whereas intra- tion with hip dysplasia and the apparent but not true in-
uterine pressure on the limbs in an atypical position can lead equality of leg length correlate with the compressed fetus
to dysplasia or hip joint dislocation. Thus a thigh held in syndrome as Browne (1965) and Dunn (1976a, 1976b)
abduction or adduction for too long late in gestation pre- reported. The pelvic tilt, hip dysplasia, and fetal postural
cedes limited mobility of hip joints and eventual pelvic scoliosis seem to develop concomitantly. Although abduc-
asymmetry (Cozen, 1968). Salter et al. (1963) reported that tion contracture of one hip can self-correct in physically
in newborn piglets hip joints maintained in extension for 8 active children, the contralateral dysplastic hip on the con-
weeks developed progressive femoral head dysplasia and vex side would take longer to rectify. A little known com-
slight subluxation. This was reversed after the piglets ran ponent of the fetal compression syndrome is the involve-
freely for 12 weeks. The authors concluded that in humans ment of the shoulders for they too are likely to be
dysplastic hip instability and even subluxation may result compressed and immobile in late pregnancy (Tönnis, 1968).
from compression in utero or develop postnatally when a
tightly wrapped baby is unable to move the legs freely (e.g.,
Navajo infants) (Coleman, 1968). Prognosis and progression
When one hip is dislocated, the other may exhibit vary-
ing degrees of dysplasia. Karski (2000) attributes most cases James (1951) divided infantile scoliosis into resolving
of idiopathic scoliosis to abduction contracture of the right (36%) and progressive (64%) types conceding inability to
hip. Unilateral congenital hip dislocation is more commonly differentiate the two with any certainty (James et al., 1959).
left-sided (Watson, 1971) and varying degrees of hip joint Nor do all in the resolving group resolve permanently. Of 86
subluxation or instability are frequent. Weissman (1954) cases of infantile scoliosis attending a clinic in the first three
contended that in infants with abduction contracture, the years of life, 24 were considered to be resolving, and 19
contralateral hip was often accompanied by hip dysplasia (a (curves ranging from 10 to 50°) were stable at that time. The
forerunner of dislocation) with adduction contracture. Co- remaining 43 infants (50%) with single or double curves all
existing changes in the two hips were considered to be progressed substantially (33 requiring surgery) during sur-
interrelated as both thighs are directed to the same side and veillance (Thompson and Bentley, 1980). Browne (1965)
associated with pelvic obliquity, the abducted leg appearing contended that congenital postural scoliosis, if not treated
to be longer (Wiseman, 1954) as the unstable head of the early after birth, progressed to severe intractable deformity
opposite femur slips out from under the cartilagenous rim of with dwarfism.
the acetabulum. Unless treated this readily missed joint Most juvenile scoliotics progress and have a bad prog-
instability can lead to pelvic asymmetry (Hart, 1942). nosis (James, 1954a, 1954b). Nash (1980) said that rapidly
Lloyd-Roberts and Pilcher (1965) reported that 10% of their growing prepubertal scoliotic children have a 60% chance
infants with compression scoliosis had pelvic obliquity, the of progression. Tolo and Gillespie (1978) reported progres-
pelvis following the curve of the trunk and being higher on sion in 71% of juvenile scoliotics, although lack of progres-
the concave side. Limited hip abduction was usually found sion does not equate with regression. Untreated, scoliotic
on the convex side, the authors believing this to be a man- juveniles progressed at a median rate of 6° per annum. In
ifestation of the compressed fetal syndrome. another 98 juvenile scoliotics 7 resolved during the follow-
Dickson (1983, 1984) reported a pelvic tilt in 40 to 50% up, 8 remained stationary, and the remainder (85%) pro-
of adolescent scoliotics. Although the spine would be de- gressed, 56% requiring spinal fusion (Figueiredo and James,
stabilized, he regarded the deformity as inconsequential 1981). In another 109 juveniles (Robinson and McMaster,
328 W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335

1996) with idiopathic scoliosis, 95.4% were progressive. correction and spinal fusion (Lonstein, 1994). For pediatric
The remaining five received brace therapy and the curves scoliosis the management generally advocated is periodic
regressed to less than 10°, one with an original angle of 40°. surveillance at variable intervals with radiological assess-
The ominous prognosis and the widely recognized propen- ment until curves are sufficiently large to warrant bracing.
sity for severe deformity (Dobbs and Weinstein, 1999; Ep- No other effective nonoperative treatment is available
stein, 1976; Morin, 1999) justify concern over the absence (Dickson, 1996).
of a satisfactory therapeutic program for juvenile scoliotics Exercise programs recommended for juvenile scoliotics
who remain vulnerable to progression during the pubertal with large curves have, in general, proved ineffective (Nash,
growth spurt. Curve regression to below 10° does not ensure 1980). Hungerford (1975) vigorously condemned exercises
lack of progression at puberty or later. alone. With few exceptions description of the physical ex-
The pathogenesis of the progression, irrespective of the ercises is not provided. Whether the exercises are directed at
initial cause, can be attributed to the unavoidable repetitive reducing the curve, or compensating for inactivity of the
biomechanical stresses of daily living applied unremittingly relatively fixed trunk in the brace for most of the day, is
and asymmetrically to the spinal deformity with its mala- unstated. Bjure et al. (1969) had recommended exercise on
ligned vertebrae and discs. As with joints elsewhere the a bicycle ergometer, jumping up and down, throwing med-
spinal constituents of bone, cartilage, and ligaments mani- icine balls, running on the spot, skipping, Indian jump, and
fest increased vulnerability to fatigue-induced degenerative bicycling, the purpose being to enhance a subject’s general
osteoarthrosis involving cumulative, molecular scissions of physique and vital capacity. However because it is impos-
macromolecules and polymers rather than assumed enzy- sible to prevent young children from running and playing, it
matic self-destruction (Stehbens, 2002). Molecular disinte- is of the utmost importance that parents maintain close
gration accounts for the degenerative changes, laxity (and surveillance with avoidance of repetitive jumping up and
tears) of ligaments, and augmentation of the architectural down movements which are detrimental and cumulatively
deformity as in blood vessels (Stehbens, 1997). aggravate scoliosis. They are akin to impact loading the
vertebrae and disks vertically and asymmetrically, thereby
increasing the flexure and shortening the fatigue life of
Current management of scoliosis spinal constituents (Stehbens, 2002).
The Schroth technique is an intensive course of in-pa-
Although conservative management of scoliosis has en- tient physiotherapy (4 – 6 weeks) for those 8 to 70 years of
compassed physiotherapy, electrical muscle stimulation, age. For 6 – 8 h/day patients are taught specific breathing
traction, and bracing, the aim here is not to appraise the use exercises and asymmetrical postural exercises promoting
of orthotics. It is to consider conservative management in body symmetry to correct the scoliosis and visible deformi-
the early stages of the deformity in young pediatric subjects. ties (Lehnert-Schroth, 1992; Weiss, 1991, 1992). Mirrors
Waiting for scoliosis progression to attain a specified degree monitor the correction and posture and physiotherapists
of severity has to be an undesirable management procedure. monitor compliance and progress. Some patients receive
Dickson (1985, 1999) maintains that if a deformity is ac- electrical muscle stimulation and orthoses. In a 31-month
ceptable at presentation, then preservation of this degree of follow-up, scoliosis was stabilized in two-thirds, progressed
acceptability is the aim. Acceptability to those resigned to in 16.1% and in 15.2% had improved more than 5° with
severe progressive deformity and spinal osteoarthrosis with long-term effects uncertain. The prime benefit seems to
greatly restricted employment and a shortened lifespan from derive from the improved vital capacity of 18% and stretch-
cardiopulmonary disease is far from optimal management ing exercises (Weiss, 1991). Ferraro et al. (1998) also re-
which should be based on an understanding of the patho- ported improvement and less progression in scoliotics given
genesis of the progression (Stehbens, 2002). active postural correction exercises of different types over
Boachie-Adjei and Lonner (1996) recommend close an average of 2 years. Sustained patient compliance was a
monitoring of pediatric scoliotics at 4 to 6 monthly intervals major problem and long-term benefit uncertain. The con-
for curvatures up to 20°. Orthoses are considered for those sensus is that exercise programs have not provided signifi-
in the range of 20 to 29° with progression of 5° and for any cant benefit (Rowe et al., 1997; Stone et al., 1979) and the
curvature of 30° or more in the skeletally immature with exercises are often detrimental. Nor has chiropraxis any-
surgery proposed for deformities of at least 40°. In the thing expedient to offer (Lantz, 2001).
absence of any therapy for children with curves up to 20°, Longitudinal traction (as on a rack) has been used in
school screening programs have fallen by the way side. scoliosis since the times of Hippocrates (Roaf, 1966). On
Galasko (1998) recommends that in general terms, idio- current evidence it has proven to be a successful temporary
pathic adolescent scoliosis is “treated” by regular observa- maneuver in severe deformities prior to bracing or surgical
tion until skeletal maturity if the curve is less than 25 to 30°. fixation (Wilkins and MacEwen, 1977). It has been applied
Moderate curves of 30 to 45° are frequently treated by by means of halotraction (halo-femoral or halo-pelvic) par-
bracing until skeletal maturity, whereas in a growing ado- ticularly in severe scoliosis (Bradford et al., 1975), the halo
lescent curves greater than 40 – 45° often require surgical being fixed to the skull and femoral or pelvic traction at the
W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335 329

lower end with the traction effected by weights. Given the bioengineering fatigue-induced severe accelerated osteoar-
serious and at times fatal complications (lesions of cranial throsis (Stehbens, 2002). The acquired laxity of the weak-
nerves and brachial plexus, respiratory problems, abnormal- ened ligaments and degeneration and deformity of the discs
ities of swallowing), all traction must be applied with cau- and vertebrae associated with biomechanical fatigue poten-
tion particularly in the presence of neurological disease. tiate further curve progression. In essence pediatric idio-
Cotrel traction (Cotrel et al., 1980; Hancox, 1981) con- pathic scoliosis management aims to (i) detect the deformity
sists of horizontal stretching of the child’s body day and at its earliest stage to initiate regression as soon as possible,
night by means of a pelvic corset attached to scales at the (ii) be aware of and to minimize underlying mechanical
bottom of the bed and a head halter with a 2-kg weight stresses responsible for progression and limit the develop-
attached to the bed top through a pulley system. Additional ment of osteoarthrotic degenerative changes, (iii) strengthen
self-imposed dynamic traction is achieved by means of a bones, ligaments, and surrounding muscles symmetrically
handle bar and stirrups attached to the static traction appa- by exercise using the spine in the manner for which it was
ratus, self-elongation by arm pressure proving more effec- designed, and (iv) encourage and sustain patient persever-
tive than foot pressure on stirrups. Intermittent pressure is ance with the ameliorative program.
gradually increased in frequency up to 30 times per hour for Bones are living tissues and readily reshaped, indented,
9 –10 h per day with unspecified exercise sessions and time or even eroded mechanically by pulsating arteries or aneu-
for schooling. The regimen continues for 7 to 10 days with rysms. Skull shape can be mechanically modified in utero or
radiological assessment of improvement. In 19 cases cor- postnatally e.g., by plagiocephaly or hydrocephalus, and
rection was over 40% and used preoperatively, the proce- elongated by infant head bandaging by Chinook Indians
dure obtains maximum possible correction. Cotrel (1965) (Dunn, 1969a, b). Tight bandaging of the feet of Chinese
achieved a curve reduction from 73° at 3 years 10 months to women produced gross distortion. Giraffe-necked Padang
54° at 5 and a half years prior to surgery. The technique women (Roaf, 1966) were subjected to deforming pressure
induces some vertebral derotation and disadvantages are the (up to 30 pounds weight) on the shoulder girdle to incor-
prolonged periods of immobilization and ensuing muscle porate thoracic vertebrae into the neck. Valgus deformity of
atrophy. big toes with eventual deformation (bunions and osteoar-
Vertical gravitational traction has been achieved by sus- throsis) can result from poor shoe design. Once established,
pending children upside down by the feet while a pelvic correction of joint deformities by physiological means be-
brace with bars is held by the hands (Majoch, 1981). Treat- comes increasingly difficult. However, plagiocephaly can
ment duration was increased from 12 min to 1 h daily and be modified or induced postnatally and in neonates some
the loading on the head was increased up to 25% of the body congenital postural and compressive deformities of the feet
weight for 4 weeks preoperatively and combined with mus- and hip dislocations can be rectified or improved by non-
cle massage, manual curve correction, and swimming to surgical orthopedic methods. Similarly in orthodontics
improve lung capacity. The program was not applied to thumb sucking deforms the palatine arch and the position of
children with hypertension or a spinal or neurological dis- teeth is dependent on the balance of opposing tongue and lip
order. A curve of 117° in a 12-year-old boy was reduced to pressures (Proffit, 2000). Permanent teeth are more easily
57° in 4 weeks and results were considered better than with moved by continuous direct pressure from a dental brace to
horizontal traction. Tabjan and Majoch (1980) used vertical improve the alignment and bite in young children whereas
traction 6 times per week for 3 to 6 weeks preoperatively, long-established anatomical deformities are less amenable
the patient hanging from parallel bars by the legs and feet to correction. The recommendations borne out by amelio-
with additional weight or traction applied to the neck. This ration of dental malalignment at an early age are relevant to
method improved respiratory capacity and allowed better scoliosis. The pressures exerted in orthodontics are gradual,
corrective force on the spine despite its discomfort to the persistent, and directly applied, whereas orthotic pressures
feet and legs. Thus, despite its hazardous and unphysiolog- brought to bear in scoliosis are of necessity indirect, not
ical application, traction remains the only technique known applied directly to the spine itself, and can cause secondary
to reduce the curvature. defomity of the rib cage. The spine and its ligaments con-
tinue to respond to prevailing stresses and mesenchymal
cells are dependent on and respond to mechanical stresses,
Rationale of management advantageous or otherwise, during growth and develop-
ment. Thus the aim should be to detect scoliosis early while
Management of juvenile scoliosis should be based on the the spine is growing and maturing and more readily re-
concept that it is not a specific disease but a mechanical shaped to a near normal configuration before the develop-
deformity of the spine complicating many diseases and that ment of serious spinal deformation and osteoarthrosis.
the most common idiopathic group comprises instances of In an indefinite number of young subjects, scoliosis re-
an habitual posture established in utero or postnatally and gresses spontaneously, with both the incidence of and the
perpetuated biomechanically (Stehbens, 2002). Irrespective explanation for regression as yet unknown. The need to
of the cause, progression of scoliotic curves results from determine the factors responsible for such regression and to
330 W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335

assess the extent of residual curvature and the nature of Therapeutic trial
long-term clinical sequelae in such subjects is obvious.
Unrecognized physical activity seems to potentiate early The boy, the youngest of three children, was induced at
regression and males with idiopathic scoliosis are more 38 weeks due to toxemia of pregnancy, associated with
inclined to regression than females. This suggests that the oligohydramnios and diminished frequency of kicking. He
unidentified physical activity is advantageous although me- grew, developed well, and was active with no family history
chanical stresses in males are likely to be greater. of scoliosis. At the end of winter when aged 4 years 10
Increased awareness of congenital postural scoliosis months his asymmetrical stature in the bath was noticed. It
(Browne, 1956, 1965) and the need for its early detection became more obvious within 2 weeks and attempts to in-
and treatment with a splint similar to Browne’s design duce him to stand straight were unsuccessful. His chest was
permitting continued mobility while correcting the curve asymmetrical with the left scapula higher and more prom-
would seem worthwhile even if some curves regress spon- inent than the right. A prominent gap was noticeable be-
tween his left arm and hip. His chest protruded slightly on
taneously. Browne’s success rate for treatment in the first 6
the left side and appeared slightly twisted. Thick clothing in
months was 100%. Conversely Walker (1965), assessing
the colder months of the year had apparently masked the
Browne’s splint for infantile scoliosis, concluded that treat-
abnormality and rapidity of the vertebral change. The
ment benefit did not differ from that of untreated cases.
child’s grumpiness and lethargy had been attributed to a
However, neither his therapy nor patient group were com- growth spurt in excess of his peers. He had occasional pain
parable to Browne’s. Even if 90% of early congenital pos- over the lower left thorax. Scoliosis was confirmed by X-ray
tural scoliotics self-correct, when and how they were man- (Fig. 1). During the 3 weeks wait to see an orthopedic
aged are unknown. The problem of treating young children surgeon, his deformed stance became even more pro-
with “intractable scoliosis,” if spontaneous regression does nounced. The two orthopedic surgeons consulted found no
not eventuate, is such that it would be wiser to treat all neurological abnormality or underlying disease. They found
affected babies within the first 6 months of life as Browne equal leg lengths and normal flexibility and movement of
recommended. There is also a need to search for dysplasia his head and neck. Radiologically there was mild thoraco-
and abduction and adduction contractures of the hip and lumbar scoliosis, convex to the left (within the range of
stiff shoulder joints and to treat the infants by early postural 15°–20°). The apex was at the T12-L1 level and no abnor-
correction (Green and Griffin, 1982). Ceballos et al. (1980) mality was detected by magnetic resonance imaging. An-
were of the opinion that treatment is imperative as soon as ticipating that the curvature would regress spontaneously,
the diagnosis of progressive scoliosis is made. They used a one surgeon recommended surveillance at 6 monthly inter-
plaster “shell” to overcorrect the lateral spinal curvature vals and the other at 12 monthly intervals. This was at
rather than Browne’s padded metallic splint. The plaster variance with the progression over previous weeks and the
shell was extended to maintain flexure and moderate abduc- proven adage “as the wind blows, so the tree grows.”
tion of the thighs for hip dysplasia. One of us (W.E.S.) recalled that in the late 1940s, trac-
For older infants and juveniles traction can be applied by tion, a body cast, and physiotherapy were utilized. Because
holding and gently swinging the child by the arms as if of concern that the curve would continue to progress and
playing. This activity could be useful until a jungle gym is become increasingly difficult to correct during prolonged
available and manageable by the child. As Browne (1956, surveillance, it was reasoned that traction was the only way
1965, 1969) emphasized, it is important to induce the child abnormal spinal curvatures (with rotation and whether sin-
gle or double) could be corrected. We conceived of exer-
to use its back in the manner appropriate for its design (i.e.,
cising the child on a jungle gym (monkey bars). This me-
with symmetrical movements) and to discourage move-
tallic frame resembles a horizontal ladder suspended well
ments or postures perpetuating the curvature. Passive but
off the ground with steps at either end for access; the child
gentle manipulation to slowly overcome the resistance to
hanging by his hands from the cylindrical rungs swings
turn and bend toward the convexity could be useful. Like- from rung to rung (back and forth). Soon adept at this, he
wise infants with a pelvic tilt, dysplasia of the hip joint, and played on the bars several times per day with alacrity, the
adduction or abduction contracture of a hip could undergo activity proving to be enjoyable for him and his siblings.
gentle passive manipulation of the thigh to overcome the The swinging motion applied the stresses equally to both
joint stiffness. Browne’s curved splint (1956, 1965) would sides of his body, the weight of his lower body providing the
also benefit both dysplastic hips. Hip dysplasia in infants traction. Improvement was rapid and noticeable within 10
usually disappears after use of an abduction splint for 6 weeks (Fig. 2). To reinforce the regression already
months (Weissman, 1954). Long-term follow-up of un- achieved, further exercises were introduced from time to
treated hip dysplasia in the Navajo reveals spontaneous time. The rationale was that physiological traction and care-
improvement by the time of skeletal maturity with only fully selected exercises would potentiate regression of the
subtle radiological changes in 40% of the hips (Schwend et curvature, strengthen the muscles and ligaments about the
al., 1999). spine simultaneously increasing vertebral bone strength and
W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335 331

such contracture was detected and the pelvic tilt, although


still present in an X-ray one year later, was less pronounced
(Fig. 3). Plagiocephaly, also associated with the syndrome
(Ceballos et al., 1980; Wynne-Davies, 1975), was absent.
Even after improvement in the scoliosis, on standing upright
the child suddenly and subconsciously assumes the pelvic
tilt with a sudden jerk reminiscent of Coleman’s description
(1968) as the femoral head slips over the posterior acetab-
ular ring (the “jerk” of exit and entry). Significantly this is
now not nearly so pronounced. The observation is consis-
tent with hip dysplasia which seems to be more important
than the contralateral abduction contraction emphasized by
Karski (2000). The limited mobility of the child’s left arm
(possibly adduction contracture) became obvious when he
attempted to swim freestyle. Unequal mobility of all limbs
had already been recognized as a component of the fetal
compression phenomenon (Tönnis, 1968).
The pelvic tilt drew attention to the difference in the
sacroiliac (SI) joints which had been overlooked and the
possibility of left hip dysplasia. On consultation with a
radiologist, computerized tomography was performed 5
months after the X-ray in Fig. 3 because it was thought that
rotation might be responsible for the difference in the SI
joints. This revealed that the legs were of equal length and
the nonossified cartilaginous gap in the anterior portion of
the right joint was 2 mm wider than that of the left. The
more posterior portions were symmetrical. Overall the im-
provements in the pelvic tilt, the SI joints, and rib displace-
ment with a reduced list of the torso and loss of the gap
between the left hip and the elbow indicate that they oc-
curred simultaneously with reduction in the scoliosis. The
boy can now stand on his left leg unassisted. The narrowing
of the left SI joint, which has not previously been observed
in the fetal compression syndrome, is likely to be due to
asymmetrical pressures on the hips.
Little information could be found on therapy for juve-
niles although physiotherapy combining passive manipula-
tion and exercises emphasizing lateral abduction of the hip
Fig. 1. This X-ray, taken 2 days after his fifth birthday, demonstrates the and shoulder is currently meeting with some success in this
left thorocolumbar scoliosis. Another X-ray demonstrated that the 12th rib juvenile. In view of the absence of an abnormal gait in this
was deflected downward more so than the right. The pelvic tilt to the left child and the radiological and visual improvements
and the narrowing of the nonossified cartilaginous gap in the left sacroiliac
joint were overlooked.
achieved, the present management policy will continue. The
improvement obtained by traction postoperatively in sub-
jects with severe scoliosis provides support for his contin-
mineral density. The visual improvement prompted a com- ued use of the more acceptable jungle gym. Daily exercises
prehensive analysis of conservative therapy and additional on a jungle gym over a longer time span can only be
reinforcing exercises. beneficial and prophylactic in such children.
This subject, a tall child with a left scoliosis and a pelvic The X-ray in Fig. 3 was taken 1 year after that in Fig. 1,
tilt, conforms to the previously undetected congenital pos- both parents being pleased with the visual and radiological
tural scoliotic profile. The overlooked pelvic tilt (Fig. 1) improvement. The lateral spinal deviation was so reduced
represents a component of congenital postural scoliosis. that the radiological report indicated no “scoliosis” and hip
Mehta (1992) considered scoliosis in most juveniles to be of joints were normal. His chest expansion is now equal and
this infantile type. The curvature concave to the right is symmetrical, his left scapula is less prominent with the
associated with pelvic obliquity (Lloyd-Roberts and Pilcher, overall cosmetic appearance vastly improved and with no
1965) and abduction contracture of the hip in at least 10%. recurrence of chest pain. Whether the displaced left 12th rib
When this possibility was perceived in the present case, no (Fig. 3) will resume a more normal position is unknown at
332 W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335

Fig. 2. Photographs taken 10 weeks after purchase and regular use of a jungle gym. (A) The distinct list to the left and asymmetrical stance with a prominent
gap between the elbow and hip on the left side. (B) Black ink dots indicate the tips of the vertebral spine. The list is still clearly visible whereas the back
demonstrates improvement of the curvature. The left scapula is still elevated and more prominent than the right.

this stage. It is anticipated that sustained monitoring and primary curve with no bending to the concave side, (iii)
further exercises with symmetrical stresses on the spine and lying supine with hips and knees flexed and abducting and
hips will improve his habitual stance. adducting both hips against moderate resistance provided by
a parent, (iv) touching the toes of the left foot with legs wide
apart in sitting and standing position, (v) lying supine while
Recommended exercises peddling in the air, and (vi) deep breathing exercises with
and without slight restrictive parental hand pressure over the
The child, now aged 6 years 8 months, is probably an lower right side of the chest to facilitate maximum left side
example of congenital postural scoliosis and was therefore expansion of the thorax.
destined to severe deformity. The historic concepts of pro- Lying supine on a firm surface while simultaneously
longed traction outlined above were drastic. The exercise performing exercises tends to exert pressure on the promi-
program and frequent use of the jungle gym led to cessation nent ribs on the convex side posteriorly. As subjects be-
of progression and distinct improvement within 10 weeks. comes older and stronger, chin-ups should be included and
This physiological, more natural use of traction, where the some simple gymnastic exercises on parallel bars or rings to
lower half of the body provides the traction, is preferable to manipulate the legs always omitting the final jarring jump
a vertical or horizontal rack with immobilization. It is ac- off the apparatus. Scoliotics under treatment should avoid
ceptable to the child and has benefited the hip joints and all strenuous exercises such as jogging, jumping, hopping,
pelvic tilt. Good nutrition being essential, this child is given skipping, marching, tramping, weight lifting, and any vibra-
a traditional high protein diet with meat, eggs, milk, and a tional exposure (tractors, bumpy roads, horse riding, pogo
variety of fruit and vegetables (Stehbens, 1993). sticks, trampolines) likely to result in vertical impacts trans-
Other regular and varied short periods of daily exercises missable to the spine. Habitual use of a sponge rubber
include (i) push-ups, (ii) repeated lateral flexion of the trunk cushion when in a car is also recommended.
to the left (convex side) when sitting, standing, or supine The angle of curvature diminishes by a mean of 9° in the
which tends to reverse vertebral torsion and reduce the supine position, the difference in 93% of subjects so exam-
W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335 333

idiopathic scoliosis is likely to be postural in nature. Older


infants, juveniles, and even adolescents with scoliosis could
be candidates for similar management in hopeful anticipa-
tion of benefit at the earliest stage rather than waiting until
bracing or surgery is mandatory. For patients with advanced
scoliosis (severe angulation or double curves) exercises
should probably be limited or avoided until the angle is
much reduced by the mild persistent traction effected by a
jungle gym or parallel bars.

Discussion and conclusion

Routine examination of neonates and young infants for


compressive postural spinal deformities and appropriate
treatment should be routine. Obstetricians, midwives, pedi-
atricians, and general practitioners should be trained in their
early detection. School screening could be reintroduced and
appropriately trained personnel could detect early curva-
tures, advising teachers and parents to routinely encourage
children to spend time on the jungle gym which should be
standard playground equipment in primary schools with
such traction activity also of benefit to those with lordosis or
kyphosis.
Since mechanical stresses of everyday life are the dom-
inant force responsible for progression, it is logical to de-
duce that as in orthodontics, counteracting forces can in-
hibit, ameliorate or cause curvature regression. Longitudinal
traction must be the mainstay of management and is the
only mechanism known to regularly reduce scoliotic curva-
tures. The logic of this exercise program is consistent with
the pathogenesis of the deformity (Stehbens, 2002) and the
evidence provided indicates that it is likely to benefit those
with early scoliosis. The overall aim of pediatric scoliosis
management should be (1) to detect the deformity at its
earliest stage because geometrical abnormalities predispose
Fig. 3. X-ray taken two weeks prior to his 6th birthday. The radiology
to osteoarthrosis (Stehbens, 2002), (2) to induce regression
report stated that no scoliosis was present. A very slight residual curve is
centered at approximately T11. The 12th left rib is clearly deflected of any deformity, to prevent or limit progression to obviate
downward and a slight pelvic tilt remains. The difference in width of the the need for orthotics or surgery, (3) to prevent relapse and
cartilaginous gaps in the SI joints is less pronounced. ensure good compliance, (4) to prevent development of
scoliosis in the community, and (5) to increase awareness of
this deformity in the community and perinatal and pediatric
ined (Torell et al., 1986) ranging from 0 to 20°. Therefore health services. This proposed management program will
lateral flexing to the convex side may be best performed hopefully restore the vertebrae and intervertebral discs to a
when supine. With the added buoyancy of the body swim- more appropriate anatomical position, shape, and more bal-
ming exercises to strengthen the limbs and trunk muscles anced mechanics and should then consolidate their retention
equally on both sides would be beneficial. Breast stroke of the normal status.
swimming and sitting cross-legged on the floor while
watching television may be of particular benefit for this
child. Continued monitoring of the subject to maintain an
exercise program during adolescence and after bone matu- Acknowledgments
ration is indicated, although older children are more able to
understand the need for self-correction. The Wellington Medical Research Foundation provided
This analysis and the proposed exercise program are financial support and Dr. G.T. Jones assisted with the print-
primarily concerned with young pediatric subjects whose ing of Fig. 3.
334 W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335

References James, J.I.P., 1951. Two curve patterns in idiopathic structural scoliosis.
J. Bone Joint Surg. 33B, 399 – 406.
Bjure, J., Grimby, G., Nachemson, A., Lindh, M., 1969. The effect of James, J.I.P., 1954a. Idiopathic scoliosis. The prognosis, diagnosis, and
physical training in girls with idiopathic scoliosis. Acta Orthop. Scand. operative indications related to curve patterns and the age at onset.
40, 325–333. J. Bone Joint Surg. 36B, 36 – 49.
Boachie-Adjei, O., Lonner, B., 1996. Spinal deformity. Pediatr. Clin. North James, J.I.P., 1954b. Prognosis and treatment of idiopathic scoliosis.
Am. 43, 883– 897. J. Bone Joint Surg. 36B, 685– 686.
Bradford, D.S., Moe, J.N., Winter, R.B., 1975. Scoliosis, in: Rothman, James, J.I.P., 1965. Classification and Prognosis, in: Zorab, P.A. (Ed.),
R.H., Simeone, F.A. (Eds.), The Spine, Vol. 1. Saunders, Philadelphia, Proceedings of a Symposium on Scoliosis, Brompton Hospital, Lon-
pp. 271–385. don, pp. 11–17.
Browne, D., 1936. Congenital deformities of mechanical origin. Proc. R. James, J.I.P., Lloyd-Roberts, G.C., Pilcher, M.F., 1959. Infantile structural
Soc. Med. 28, 1409 –1431. scoliosis. J. Bone Joint Surg. 41B, 719 –735.
Browne, D., 1956. Congenital postural scoliosis. Proc. R. Soc. Med. 49, Karski, T., 2000. The Etiology of the So-Called Idiopathic Scoliosis
395–398. Wydawnictivo KGM, Lublin, 2000.
Browne, D., 1965. Congenital postural scoliosis. Br. Med. J. ii, 565–566. Lantz, C.A., Chen, J., 2001. Effect of chiropractic intervention on small
Browne, D., 1969. Infantile Scoliosis. Clin. Proc. Children’s Hosp. Dist. scoliotic curves in younger subjects: a time-series cohort design. J.
Columbia 25, 157–177. Manip. Physiol. Ther. 24, 385–393.
Ceballos, T., Ferrer-Torrelles, M., Castillo, F., Fernandez-Paredes, E., Lehnert-Schroth, C., 1992. Introduction to the three-dimensional scoliosis
1980. Prognosis in infantile idiopathic scoliosis. J. Bone Joint Surg. treatment according to Schroth. Physiotherapy 78, 810 – 821.
62A, 863– 875. Lloyd-Roberts, G.C., Pilcher, M.F., 1965. Structural idiopathic scoliosis in
Coleman, S.S., 1968. Congenital dysplasia of the hip in the Navaho infant. infancy. A study of the natural history of 100 patients. J. Bone Joint
Clin. Orthop. 56, 179 –193. Surg. 47B, 520 –523.
Cotrel, Y., 1965. Conservative Management, in: (Zorab, P.A., Ed.), “Pro- Lonstein, J.E., 1994. Adolescent idiopathic scoliosis. Lancet 344, 1407–
ceedings of a Symposium on Scoliosis” Brompton Hospital, London, 1412.
pp. 18 –20. Majoch, S., 1981. Gravitational traction in preparation for surgery. Phys-
Cotrel, Y., Seringe, R., Plais, P.Y., Paluzak, M., 1980. Spinal Traction in iotherapy 67, 72–73.
Scoliosis, in: Zorab, P.A., Siegler, D. (Eds.), Scoliosis 1979. Academic Mehta, M.H., 1992. The conservative management of juvenile idiopathic
Press, London, pp. 241–261. scoliosis. Acta Orthop. Belg. 58 (Suppl. 1), 91–97.
Cozen, L., 1968. Some evils of fixed abduction of the hip. Clin. Orthop. 57, Morin, C., 1999. Traitement des scolioses idiopathiques chez l’enfant en
203–211. période de croissance. Bull. Acad. Natl. Méd 181, 731–735.
Dickson, R.A., 1983. Scoliosis in the community. Br. Med. J. 286, 615– Nash, C.L., 1980. Current concepts review. Scoliosis bracing. J. Bone Joint
618. Surg. 62A, 848 – 852.
Dickson, R.A., 1984. Screening for scoliosis. Br. Med. J. 289, 269 –270. Proffit, W.R., 2000. Contemporary Orthodontics, third ed. Mosby, St.
Dickson, R.A., 1985. Conservative treatment for idiopathic scoliosis. Louis, pp. 128 –135.
J. Bone Joint Surg. 67B, 176 –181. Roaf, R., 1966. Scoliosis. Livingstone, Edinburgh.
Dickson, R.A., 1996. Spinal Deformities in Children, in: Duthie, R.B., Robinson, C.M., McMaster, M.J., 1996. Juvenile idiopathic scoliosis.
Bentley, G. (Eds.), Mercer’s Orthopaedic Surgery, ninth ed. Arnold, J. Bone Joint Surg. 78A, 1140 –1148.
London, pp. 475–548. Rowe, D.E., Bernstein, S.M., Riddick, M.F., et al., 1997. A meta-analysis
Dickson, R.A., 1999. Spinal deformity—adolescent idiopathic scoliosis. of the efficacy of non-operative treatments for idiopathic scoliosis.
Nonoperative treatment. Spine 24, 2601–2606. J. Bone Joint Surg. 79A, 664 – 674.
Dickson, R.A., Stamper, P., Sharp, A-M., Harker, P., 1988. School screening Salter, R.B., Kostvik, J., Schatzker, J., 1963. Experimental dysplasia of the
for scoliosis: cohort study of clinical course. Br. Med. J. ii, 265–267. hip and its reversability in newborn pigs. J. Bone Joint Surg. 45A,
Dobbs, M.B., Weinstein, S.L., 1999. Infantile and juvenile scoliosis. Or- 1781.
thop. Clin. North Am. 30, 331–341. Schwend, R.M., Pratt, W.B., Fultz, J., 1999. Untreated acetabular dysplasia
Dunn, P.M., 1976a. Congenital postural deformities. Br. Med. Bull. 32, of the hip in the Navajo. Clin. Orthop. 364, 108 –116.
71–76. Stehbens, W.E., 1993. The Lipid Hypothesis of Atherogenesis. Austin,
Dunn, P.M., 1976b. Perinatal observations on the etiology of congenital Landes.
dislocation of the hip. Clin. Orthop. 119, 11–22. Stehbens, W.E., 1997. The pathogenesis of atherosclerosis: a critical re-
Epstein, B.S., 1976, fourth ed. Lea & Febinger, Philadelphia, pp. 216 –220. view of the evidence. Cardiovasc. Pathol. 6, 123–153.
Ferraro, C., Masiero, S., Venturin, A., Pigatto, M., Magliorino, N., 1998. Stehbens, W.E., 2003. Pathogenesis of idiopathic scoliosis revisited. Exp.
Effect of exercise on mild idiopathic scoliosis. Eur. Medicophys. 34, Mol. Pathol. 74, 49 – 60.
25–31. Stone, B., Beekman, C., Hall, B., Guess, V., Brooks, H.L., 1979. The effect
Figueiredo, U.M., James, J.I.P., 1981. Juvenile idiopathic scoliosis. J. Bone of an exercise program on changes in curve in adolescents with min-
Joint Surg. 63B, 61– 66. imal idiopathic scoliosis. Phys. Ther. 59, 759 –763.
Galasko, C.S.B., 1998. Spinal Problems in Children, in: Maddiso, P.J., Tabjan, W., Majoch, S., 1980. Gravitational Traction, in: Zorab, P.A.,
Isenberg, D.A., Woo, P., Glass, D.N. (Eds.), Oxford Textbook of Siegler, D. (Eds.), Scoliosis 1979, Academic Press, London, pp. 233–
Rheumatologyn, Vol. 1. Oxford University Press, Oxford, pp. 131–134. 240.
Green, N.E., Griffin, P.P., 1982. Hip dysplasia associated with abduction Thompson, S.K., Bentley, G., 1980. Prognosis in infantile idiopathic sco-
contracture of the contralateral hip. J. Bone Joint Surg. 64A, 1273– liosis. J. Bone Joint Surg. 62B, 151–154.
1281. Tolo, V.T., Gillespie, R., 1978. The characteristics of juvenile idiopathic
Hancox, V., 1981. Cotrel traction for patients with scoliosis. Physiotherapy scoliosis and results of its treatment. J. Bone Joint Surg. 60B, 181–188.
87, 71–72. Tönnis, D., 1968. The correlation between oblique deformities and infan-
Hart, V.L., 1942. Primary genetic dysplasia of the hip with and without tile scoliosis. J. Bone Joint Surg. 50B, 881.
classical dislocation. J. Bone Joint Surg. 24, 753–771. Torell, G., Nachemson, A., Haderspeck-Grib, K., Schultz, A., 1986. Stand-
Hungerford, D.S., 1975. Spinal deformity in adolescence. Early detection ing and supine Cobb measures in girls with idiopathic scoliosis. Spine
and nonoperative management. Med. Clin. North Am. 59, 1517–1525. 11, 425– 427.
W. E. Stehbens, R. L. Cooper / Experimental and Molecular Pathology 74 (2003) 326 –335 335

Walker, G.F., 1965. An evaluation of an external splint for idiopathic Weissman, S.L., 1954. Congenital dysplasia of the hip. Observations on the
structural scoliosis in infancy. J. Bone Joint Surg. 47B, 524 –525. “normal” joint in cases of unilateral disease. J. Bone Joint Surg. 36B,
Watson, G.H., 1971. Relation between side of plagiocephaly, dislocation of 385–396.
hip, scoliosis, bat ears, and sternomastoid tumours. Arch. Dis. Child. Wilkins, C., MacEwen, G.D., 1977. Cranial nerve injury from halo trac-
46, 201–210. tion. Clin. Orthop. 126, 106 –110.
Weiss, H.R., 1991. The effect of an exercise program on vital capacity and Wynne-Davies, R., 1968. Familial (idiopathic) scoliosis. J. Bone Joint
rib mobility in patients with idiopathic scoliosis. Spine 16, 88 –93. Surg. 50B, 24 –30.
Weiss, H.R., 1992. The progression of idiopathic scoliosis under the Wynne-Davies, R., 1975. Infantile idiopathic scoliosis. Causative factors,
influence of a physiotherapy rehabilitation programme. Physiotherapy particularly in the first six months of life. J. Bone Joint Surg. 57B,
78, 815– 821. 138 –141.

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