Infantile Scoliosis

Author: Palaniappan Lakshmanan, MBBS, MS, AFRCS, FRCS (Tr & Orth),
Specialist Registrar, Department of Trauma and Orthopedics, Wansbeck General
Hospital, UK
Coauthor(s): Jeetender Pal Peehal, MBBS, MS, MRCS, Knee Research Fellow,
Positional MRI Centre, Woodend Hospital, UK; Sashin Ahuja, MBBS, FRCS, MSc,
MS, Consultant Spinal Surgeon, Department of Orthopedics, University Hospital Of
Wales, Cardiff, UK
Contributor Information and Disclosures
Updated: Jul 10, 2009

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Overview
Workup
Treatment
Follow-up
Multimedia

References
Keywords
Further Reading

Introduction
The term scoliosis is derived from the Greek word skol, meaning "twists and turns" and
refers to a sideward (right or left) curve in the spine. Scoliosis is not a simple curve to
one side but, in fact, is a more complex, 3-dimensional deformity that often develops in
childhood.

Preoperative and postoperative radiographs show an increase in the space available

for lung (SAL) after correction of scoliosis by VEPTR (vertical expandable
prosthetic titanium rib).

[ CLOSE WINDOW ]

Preoperative and postoperative radiographs show an increase in the space available

for lung (SAL) after correction of scoliosis by VEPTR (vertical expandable
prosthetic titanium rib).

Preoperative and postoperative radiographs show an increase in the space available

for lung (SAL) after correction of scoliosis by VEPTR (vertical expandable
prosthetic titanium rib).
[ CLOSE WINDOW ]

Preoperative and postoperative radiographs show an increase in the space available

for lung (SAL) after correction of scoliosis by VEPTR (vertical expandable
prosthetic titanium rib).
Recent studies
In a retrospective study of the treatment of patients with idiopathic infantile scoliosis, 31
consecutive patients (average age, 25 mo) with a primary diagnosis of idiopathic infantile
scoliosis were reviewed. Treatment modalities included bracing, serial body casting, and
vertical expandable prosthetic titanium rib (VEPTR). Of the 31 patients, 17 were treated

with a brace, 9 of whom had curve progression and subsequently received other
treatments. Of the 8 patients who responded to brace treatment, overall improvement was
51.2%. Patients who received body casts had a mean preoperative Cobb angle of 50.4
and had an average correction of 59.0%. Patients who were treated with VEPTR had a
mean preoperative Cobb angle of 90 and had an average correction of 33.8%. The study
results suggest that body casting is useful in cases of smaller, flexible spinal curves, and
VEPTR is a viable alternative for larger curves.1
Another retrospective case series, of magnetic resonance imaging (MRI) findings in
patients with presumed infantile idiopathic scoliosis, reviewed the medical records of 54
patients. MRI revealed a neural axis abnormality in 7 (13%) of 54 patients who
underwent MRI. Of these 7 patients, 5 (71.4%) required neurosurgical intervention.
Tethered cord requiring surgical release was identified in 3 patients, Chiari malformation
requiring surgical decompression was found in 2 patients, and a small nonoperative
syrinx was found in 2 patients. The authors concluded that on the basis of these findings,
close observation may be a reasonable alternative to an immediate screening MRI in
patients presenting with presumed infantile idiopathic scoliosis and a curve greater than
20.2
A recent study reviewed the frequency of asymmetric lung perfusion and ventilation in
children with congenital or infantile thoracic scoliosis before surgical treatment and the
relationship between Cobb angle and asymmetry of lung function. The authors found that
asymmetric ventilation and perfusion between the right and left lungs occurred in more
than half of the children with severe congenital and infantile thoracic scoliosis, but the
severity of lung function asymmetry did not relate to Cobb angle measurements.
Asymmetry in lung function was influenced by deformity of the chest wall in multiple
dimensions and could not be ascertained by chest radiographs alone.3

History of the Procedure

Probably the oldest mention of scoliosis is in ancient Hindu mythology (3500 to 1800
BC), in which Krishna corrects the hunchback of one of his followers. Hippocrates (460
to 377 BC) wrote about scoliosis and devices to correct it. The term infantile scoliosis
was first used by Harrenstein in 1930 and by James in 1951 in describing the clinical
entity idiopathic infantile scoliosis.4,5,6

Problem
The term infantile scoliosis is used specifically to describe scoliosis that occurs in
children younger than 3 years. Other terms for scoliosis also depend on the age of onset,
such as juvenile scoliosis, which occurs in children aged 4-9 years, and adolescent
scoliosis, which occurs in those aged 10-18 years. These terms, however, are now being
replaced by the broader terms early-onset scoliosis and late-onset scoliosis, depending on
whether the scoliosis occurs before or after 5 years of age.

In 80% of cases of scoliosis, there is no obvious cause; this is termed idiopathic scoliosis.
In the remaining 20% of cases, a definite cause can be found. These cases are divided
into 2 types: nonstructural (functional) and structural scoliosis, which could be part of a
well-recognized syndrome (syndromic scoliosis), congenital spinal column abnormalities
(congenital scoliosis), neurologic disorders, and genetic conditions.
The syndromes that can produce congenital scoliosis are VATER syndrome (vertebral
anomalies, anorectal anomalies, tracheo-esophageal fistula, and renal anomalies),
VACTERL syndrome (vertebral anomalies, anorectal anomalies, tracheo-esophageal
fistula, renal and vascular anomalies, and cardiac and limb defects), Jarcho-Levin
syndrome, Klippel-Feil syndrome, Alagille syndrome, Wildervank syndrome, Goldenhar
syndrome, Marfan syndrome, and MURCS association (M llerian, renal,
cervicothoracic, and somite abnormalities).
The congenital anomalies of the vertebral spinal column include defects of segmentation
(block vertebra, unilateral bar) and defects of formation (hemivertebra fully
segmented, semisegmented, incarcerated and nonsegmented, wedge vertebra). The
neurologic deficits in congenital scoliosis may be secondary to the spinal deformity or
may be associated with vertebral anomalies (spinal dysraphism diastematomyelia,
myelocele, myelomeningocele, meningocele). A higher incidence of idiopathic scoliosis
has been reported in families of children with congenital scoliosis. Spondylocostal
dysostosis (Jarcho-Levin syndrome) has a genetic etiology.7,8,9,10

Frequency
Infantile scoliosis is a rare condition, accounting for less than 1% of cases of idiopathic
scoliosis in North America; in Europe, the rate is 4%.
Sex: Males account for 60% of the cases of early-onset scoliosis; 90% of the cases of
early-onset scoliosis resolve spontaneously, but the other 10% of cases progress to a
severe and disabling condition. Females constitute 90% of late-onset cases and need close
monitoring to intervene at appropriate times.

Etiology
Although the exact cause of idiopathic infantile scoliosis is not known, hypotheses have
been proposed on the basis of epidemiologic evidence7,8,9,11,12 :

One theory holds that the mechanical factors during intrauterine life are
responsible for the higher incidence of plagiocephaly, developmental dysplasia of
the hip, and scoliosis on the same side of the body.
A second hypothesis suggests multifactorial causes, including predisposing
genetic factors that are either facilitated or inhibited by external factors such as
defective motor development or collagen disorders, joint laxity, and nursing
posture of the infant.

Other associations include older mothers from poorer families, breech

presentation, and premature and male low-birth-weight babies.

Pathophysiology
Most of the curves in the spine develop during the first year of life, and strong correlation
has been found between the nursing posture of the infant and development of the curve. It
is less common in the United States than in Europe, where babies are nursed in the supine
position. Infants have a natural tendency to turn toward the right side, and because of
plasticity of the infant's axial skeleton, this can lead to development of plagiocephaly, bat
ear on the right side, and curvature of the spine toward the left side.11

Presentation
Infantile scoliosis usually is detected during the first year of life either by the parents or
by the pediatrician during routine examination of the infant. Usually, a single, long,
thoracic curve to the left is present; less often, a thoracic and lumbar double curve is
noted. A child who is diagnosed with scoliosis requires a thorough clinical and radiologic
examination to exclude any congenital, muscular, or neurologic causes.

Indications
There are 3 management options for infantile scoliosis: observation, orthosis, and
operative. The decision when to use each of these is based on the rib-vertebral angle
difference (RVAD), established by Mehta in 1972 (see Image 1).13 The RVAD is a useful
guide in distinguishing between resolving and progressive idiopathic infantile scoliosis.

RVAD (rib-vertebral angle difference) measurement at apical vertebra: RVAD = b-a

(concave - convex side).
[ CLOSE WINDOW ]

RVAD (rib-vertebral angle difference) measurement at apical vertebra: RVAD = b-a

(concave - convex side).
The rib-vertebrae angle is measured by (1) drawing a line perpendicular to the middle of
the upper or lower border of the apical vertebrae of the curve and then (2) measuring the
angle this line makes with medial extension of another line drawn from the mid point of
the head to the mid point of the neck of the rib, just medial to the beginning of the shaft
of the rib. The difference between the right and the left side (concave and the convex
side) is the RVAD.
The apical vertebra is the vertebra at the curve of the apex. If there are the same number
of vertebrae between the superior and the inferior end vertebrae, there will be 2 apical
vertebrae.
For scoliosis curves with an RVAD of less than 20, observation every 4-6 months is
sufficient. If the RVAD is more than 20 or if it is not flexible clinically (ie, curve cannot

be corrected even slightly with different postures, especially lateral bending), then it is
considered to be progressive until proven otherwise.
Management with orthosis is necessary when the curve is considered to be progressive or
if a compensatory curve has developed. Various types of orthosis are available for
children younger than 3 years. The most commonly used orthoses are the hinged Risser
jacket; the plaster spinal jacket (Cotrel EDF [elongation, derotation, flexion] type)
applied under anesthesia; the Milwaukee brace; and the Boston brace. The brace should
be used for 23.5 hours a day and should be removed only for exercises and swimming. It
needs to be used until skeletal maturity is attained, because curves usually do not
progress after skeletal maturity; however, curves may progress in spite of using a
brace.14,15
Spinal deformity in scoliosis progresses during periods of peak growth velocity. The first
spinal growth peak occurs at 2 years of age, and the second peak occurs during the
prepubescent period.
Operation is usually an option only for children in older age groups (ie, around age 10
years), and segmental posterior wiring to 2 L-rods without fusion is preferable until
combined posterior and anterior fusion can be done. These procedures, however, have
been associated with complications in 50% of patients.
Because of advances in instrumentation, pedicle screw instrumentation can be performed
for children with further growth potential. In these patients, a growing rod is used,
which is associated with fewer complications than surgical fixation using L-rods. The
disadvantage associated with the growing rod is that every 6 months the posterior aspect
has to be opened to lengthen the rod, which increases the risk of infection; however, if the
curve is severe or increases despite the use of orthosis, a short anterior and posterior
fusion is recommended to prevent crankshaft phenomenon.

Relevant Anatomy
The spine is made up of 33 individual vertebrae that form a column. The spine is divided
into 5 regions, starting from the top:

Cervical - 7 vertebrae
Thoracic - 12 vertebrae
Lumbar - 5 vertebrae
Sacrum - 5 vertebrae
Coccyx - 4 vertebrae

The sacrum and coccyx are fused in the adult. The spine provides a protective function
for the spinal cord; bears and distributes the weight of the body; provides an area for
attachment of ligaments and muscles; and is the site for production of red blood cells.
Together, all the vertebrae form a flexible structure providing mobility for the body to
bend forward or sideward.

Each vertebra has a cushionlike fibrous structure called a disk, which acts like a shock
absorber during movements of the spine. The disk is made up of a soft, jellylike central
nucleus pulposus surrounded by a ring of fibrous tissue called an anulus, which is
actually a strong ligament between 2 adjacent vertebrae.
Developmentally, the spine of the fetus is C-shaped, with concavity in the front
(kyphotic) of the thoracic region; this is called the primary curve. Two secondary curves
develop after birth, with concavity occurring anteriorly (lordosis); one of the secondary
curves develops in the cervical region as the infant starts to hold up the neck, and the
second curve develops in the lumbar region when the child starts to walk. Normally, there
are no sideward (scoliosis) curves, so that the spine looks straight when viewed from
behind or from the front.

More on Infantile Scoliosis

Overview: Infantile Scoliosis
Workup: Infantile Scoliosis
Treatment: Infantile Scoliosis
Follow-up: Infantile Scoliosis
Multimedia: Infantile Scoliosis
References
Further Reading