Professional Documents
Culture Documents
01-Batra Et Al
01-Batra Et Al
From Llandough Hospital and University Hospital of Wales, Cardiff, United Kingdom
Congenital scoliosis remains an interesting and challenging diagnostic entity. Vertebral absence, partial
formation or lack of segmentation may cause asymmetrical growth and resultant deformity. Because of
the high frequency of associated anomalies within
and outside the spine, a detailed history and physical
examination are mandatory. Maternal, perinatal history, family history, and developmental milestones
must be fully explored.
Plain radiographs remain standard for diagnosis of
congenital anomalies and measuring curve magnitude, progression and perhaps growth potential of
the vertebral anomaly. Preoperative CT scan defines
the anatomy and avoids any unexpected intraoperative posterior element deficiencies. MRI can exclude
associated conditions of the spine, cranio-cervical
junction and viscera. The recognition of curves with
a bad prognosis at an early stage is pertinent to
prevent curve progression and possible neurological
complications.
The goal of surgery is to achieve a straight spine and
a physiological sagittal profile while maintaining
flexibility, to arrest progression of the curve with
a short fusion segment preserving as much normal
spinal growth as possible.
Developments in gene research continue to be
promising and may potentially lead to early detection
of congenital vertebral malformations.
BACKGROUND
Congenital scoliosis (CS) is defined as a lateral
curvature of the spine due to a developmental
No benefits or funds were received in support of this study
148
S. BATRA, S. AHUJA
CONGENITAL SCOLIOSIS
149
150
S. BATRA, S. AHUJA
CONGENITAL SCOLIOSIS
151
include dermoid cysts, epidermoid cysts, teratomas, and lipomas as well as tethered spinal cord
and they may all need treatment prior to spinal
surgery to minimise risk of subsequent neural
injury. With such a high incidence of associated
intraspinal anomalies, routine MRI screening of
patients with congenital scoliosis has been suggested (4). The specific indications for MR imaging for
possible intraspinal anomalies in congenital scoliosis include : the presence of neurological defects
such as weakness, sensory loss, bowel or bladder
dysfunction ; an associated skin abnormality over
the spine such as a dimple, hairy patches, or nevi ;
complaints of back or leg pain ; patients with lumbrosacral kyphosis ; radiographic evidence of interpedicular widening, diastematomyelia or presence
of a unilateral congenital bar with a contralateral
hemivertebra ; and any patient who is to undergo
spinal stabilisation surgery (4).
Unrecognised abnormalities of the renal system
may be present in 25 to 33% of patients and include
unilateral horseshoe kidney, renal agenesis,
duplicated kidney/ureters and hypospadias (13). All
patients with congenital scoliosis warrant a renal
evaluation by either intravenous pyelogram or renal
ultrasound/MRI.
Congenital heart disease is present in 10% of
patients, ranging from atrial and ventricular septal
defects, which are the most common abnormalities,
to complex congenital heart defects, such as
tetralogy of Fallot and transposition of the great
vessels. A screening echocardiogram and cardiologic evaluation is needed for patients undergoing surgical correction for a congenital spine
deformity.
The development of restricted pulmonary function is a concern. Recent studies imply that curve
magnitude directly affects pulmonary function, but
the development of restrictive lung function occurs
only as the curve approaches 90 (30). Severe
curves in young children tend to produce the most
severe pulmonary compromise. This restriction
may be due more to hypoplastic lung development
than to a physical constriction from the curve ;
alveolae continue to develop in the growing child
up to 8 years of age. Vital capacity screening is recommended for patients with severe curves.
Acta Orthopdica Belgica, Vol. 74 - 2 - 2008
152
S. BATRA, S. AHUJA
thoracic insufficiency syndrome (10). In young children, the Adams forward bending test (looking for
prominence of the ribs in the thoracic spine or
transverse processes in the lumbar spine) is not
possible, but the test can be simulated by laying the
child in a prone position over the knee of the examiner. Curve flexibility can be assessed by placing
the child in a lateral position over the knee of the
examiner or by suspending the infant over the arm
of the examiner.
Spinal balance in both the coronal and the sagittal planes must be evaluated. Truncal imbalance,
head tilt, shoulder inequality, and pelvic balance
should be addressed. Photographs serve as an
important aid in sequential evaluation of progress.
Given the association of neural axis abnormalities
and the possibility of neurological compromise in
congenital spine deformities, a detailed motor, sensory and reflex examination, (including abdominal
reflexes) is mandatory. Muhonen et al (29)
described the absence of an abdominal reflex as the
only objective finding seen in some patients with a
Chiari malformation. When the results of reflex
testing are abnormal, the reflex is usually absent on
the convex side of the curve.
The vital capacity of patients with congenital
scoliosis has been compared with those with idiopathic scoliosis. For any given Cobb angle the loss
in vital capacity was approximately 15% greater in
congenital compared with idiopathic scoliosis. This
greater impairment in lung function in congenital
scoliosis has been proposed to be due to the associated rib deformity or to an underlying lung anomaly (30). Vital capacity screening has been recommended for patients with severe curves. A full
spirometry work-up is recommended if surgery is
planned for those with a vital capacity < 60% of
normal (30).
RADIOGRAPHIC ASSESSMENT
Plain radiographs remain standard for diagnosis
of congenital anomalies and measuring curve magnitude, progression and perhaps growth potential of
the vertebral anomaly. Conventional radiography
often is difficult to interpret because of the patients
small size, the complex nature of the deformity,
153
CONGENITAL SCOLIOSIS
Fig. 4. Preoperative three-dimensional reconstructed computed tomography scan defines the anatomy of the curve and
may aid in the positioning of the implants.
The skill in managing a patient with a congenital spine deformity lies not only in the ability to
perform major complex salvage surgery in patients
presenting at a late stage with a severe rigid deformity, but in recognising those curves with a bad
prognosis at an early stage to prevent curve progression and possible neurological complications.
Meticulous management planning requires an
astute knowledge of the natural history of all types
of congenital spine deformity and the methods of
treatment that are available. Once high-risk anomalies such as unilateral unsegmented bars with contralateral hemivertebrae are recognised, treatment
is initiated regardless of age to prevent deformity.
NON-SURGICAL TREATMENT
Congenital vertebral anomalies can be recognised fortuitously on radiographs of infants before
clinical deformity develops. Both these and
patients diagnosed later in life should be followed
carefully at 4 to 6 month intervals until the end of
growth. The goals of observation are to recognise
the presence of vertebral anomalies, which require
immediate treatment, and to detect the progression
of curves.
Acta Orthopdica Belgica, Vol. 74 - 2 - 2008
154
S. BATRA, S. AHUJA
Bracing
Bracing plays a very limited role in the management of congenital scoliosis, especially as a primary form of treatment because these curves are typically inflexible. Less than 10% of congenital curves
can be treated by bracing. Bracing is sometimes
used to prevent progression of secondary curves
that develop above or below the congenital curve
and are causing balance problems. The Milwaukee
brace is the brace of choice for upper curves.
A TLSO (thoracic/lumbar/sacral orthosis) brace is
adequate for lumbar curves. The optimum duration
of daily brace wear is still under investigation.
As long as the curve remains controlled, treatment
should continue until skeletal maturity. The
brace may not arrest or correct development of the
curve, but it may slow progression and maintain
flexibility, allowing surgery at a later age. Bracing
serves no function in short, sharp, rigid curves.
SURGICAL TREATMENT
(4) Carefully monitored use of controlled hypotension to minimise blood loss to prevent the
occurrence of cord ischaemia, especially during any corrective manoeuvres.
(5) Motor- and sensory-evoked potential monitoring, supplemented by a wake-up test in cases
with intraoperative changes in neurological
monitoring that do not return to baseline.
(6) The postoperative monitoring of a patients
neurological status, given that paraplegia after
deformity surgery may present in a delayed
fashion, especially in the first 72 hours (28).
(7) Early and aggressive treatment of deformities
before they become severe.
The procedures are broadly divided into (a)
those preventing further deformity and (b) those
that correct the present deformity ; with the latter
type further subdivided into techniques that correct
the curve gradually and those that correct the curve
acutely.
Prevention of Future Deformity
In Situ Fusion
Principles of surgery
As a guiding principle, it has been widely recognised in studies that it is easier to prevent a deformity than allow it to progress and attempt correction at a later stage (17). The goal of surgery is to
achieve a straight spine, a physiological sagittal
profile while maintaining flexibility, to arrest progression of the curve and as short a fusion segment
as possible, preserving as much normal spinal
growth as possible. The risk of neurological injury
with surgical treatment of patients with congenital
spinal deformities is greater than in patients with
idiopathic spinal deformity (34). The occurrence of
a perioperative neurological deficit may be minimised in multiple ways by :
(1) A routine use of MRI evaluation of the spinal
cord.
(2) Earlier treatment of a spinal cord anomaly.
(3) Preventing lengthening the spinal cord intraoperatively by avoiding distraction and using
shortening procedures.
155
CONGENITAL SCOLIOSIS
156
S. BATRA, S. AHUJA
achieved acutely at the time of the initial procedure, thus postoperatively,a corrective cast is used
to encourage fusion of the spine in a somewhat corrected position, which will therefore improve the
starting point and permit the ultimate correction to
rely less on the growth of the spine The total correction obtained by performing a convex hemiepiphysiodesis varies because the younger the child at
the time of the operation, the more potential that
exists for correction over time.
Growing Rods
Single and Dual Growing Rods
The growth of the spine is greatest during the
first 5 years of life as sitting height reaches two
thirds of the adult level by age 5, while thoracic
volume reaches 30% of the adult size by five years
of age. Congenital scoliosis is associated with short
stature and diminished trunk height. Long fusions
performed on younger children may have a further
deleterious effect on trunk height and thoracic volume, leading to thoracic insufficiency. In the
absence of congenital rib fusions, early progressive
deformities may best be managed using a growing
rod technique, pioneered by Paul Harrington (43) in
the 1960s. If the child is still very young, the primary congenital curve can be treated with fusion in
situ, hemiepiphysiodesis and/or hemiarthrodesis,
excision, or osteotomy, and the corrected curve can
then be treated with a growing rod until the child is
older. This avoids fusing the entire curve, which
will lead to growth retardation and potentially
harmful pulmonary effects.
The goals of treatment have been to achieve correction of the spinal deformity without extensive
fusion, maintain correction during the subsequent
growth period, allow spinal growth and lung development, and avoid or eliminate the need for definitive fusion of the spine at an early age.
Akbarnia and McCarthy (1) reported their series
of a dual growing rod construct due to problems
associated with a single-rod implant, including
hook dislodgement and rod breakage. This is performed by subperiosteal dissection of the anchor
sites proximally and distally and by placement of
claw constructs. Rods are then placed subcutaneously on each side and joined with tandem con-
157
CONGENITAL SCOLIOSIS
158
S. BATRA, S. AHUJA
CONGENITAL SCOLIOSIS
159
160
23.
24.
25.
26.
27.
28.
29.
30.
31.
32.
33.
34.
S. BATRA, S. AHUJA
35. Roaf R. The treatment of progressive scoliosis by unilateral growth-arrest. J Bone Joint Surg 1963 ; 45-B : 637-651.
36. Ruf M, Harms J. Posterior hemivertebra resection with
transpedicular instrumentation :early correction in children aged 1 to 6 years. Spine 2003 ; 28 : 2132-2138.
37. Shands AR, Eisberg HB. The incidence of scoliosis in the
state of Delaware. A study of 50,000 minifilms of the chest
made during a survey for tuberculosis. J Bone Joint Surg
1955 ; 37-A : 1243-1255.
38. Shawen SB, Belmont PJ Jr, Kuklo TR et al. Hemimetameric segmental shift : a case series and review. Spine
2002 ; 27 : 539-544.
39. Suk S, Chung E, Lee S et al. Posterior vertebral column
resection in fixed lumbosacral deformity. Spine 2005 ; 30 :
703-710.
40. Terminology Committee of the Scoliosis Research
Society. A glossary of scoliosis terms. Spine 1976 ; 1 : 5758.
41. Winter R. Convex anterior and posterior hemi-arthrodesis
and epiphyseodesis in young children with progressive
congenital scoliosis. J Pediatr Orthop 1981 ; 1 : 361-366.
42. Winter R, Lonstein J, Davis F, de la Rosa H. Convex
growth arrest for progressive scoliosis due to hemivertebrae. J Pediatr Orthop 1988 ; 633-638.
43. Winter RB, Moe JH. The results of spinal arthrodesis for
congenital spine deformity in patients younger than
5 years old. J Bone Joint Surg 1982 ; 64-A : 419-432.
44. Winter RB, Moe JH, Eilers VE. Congenital scoliosis :
A study of 234 patients treated and untreated. J Bone Joint
Surg 1968 ; 50-A : 15-47.
45. Wynne-Davies R. Congenital vertebral anomalies :
aetiology and relationship to spina bifida cystica. J Med
Genet 1975 ; 12 : 280-288.
46. Zadeh HG, Sakka SA, Powell MP, Mehta MH. Absent
superficial abdominal reflexes in children with scoliosis.
An early indicator of syringomyelia. J Bone Joint Surg
1995 ; 77-B : 762-767.