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14 Syndromic Scoliosis
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a
Fig. 14.2 NF Type 1–associated scoliosis treated with growth-friendly instrumentation in a
6-year-old male: (a) AP view: Signicant right-sided thoracic curve and left-sided thoracic curve.
(b) Lateral view: Signicant proximal thoracic kyphosis (c) 2-year follow-up AP view: Magnetically
controlled growth rods placed with the patient receiving routine distraction every four months. (d)
2-year follow-up lateral view: Restoration of adequate thoracic kyphosis
b
c
d
14.4 Physical Examination
Physical examination for syndromic scoliosis should involve evaluation of overall
appearance, skin, and neurologic system in addition to the shape of the patient’s back
(Video 14.4). Thorough skin examination should be performed, looking for any cutaneous abnormalities such as café-au-lait spots, freckles, or neurobromas. Height
should be measured at each visit to monitor the patient’s skeletal growth. Tanner’s
pubertal development should be assessed to evaluate pubertal maturity or associated
delay. Patients with long, thin ngers and increased arm to height ratio should raise
suspicion for Marfan syndrome and warrant the additional evaluation, including cardiac imaging. Joint laxity, range of motion, and skin hyperelasticity should be noted.
A thorough neurologic examination should be performed, noting any focal neurologic decits or radicular pain. Evidence of neuropathy in combination with signicant cavus may suggest Charcot-Marie Tooth disease. When examining the back, the
presence of any hair patches or skin dimples should be noted as these may be signs
of spinal dysraphism (Chap. 31). Pelvic height should be measured for any inequality
of lower-limb length, along with any inequalities in shoulder elevation.
14.5 Radiographic Examination
Radiographic evaluation of syndromic scoliosis involves determining the severity
of the curve (Video 14.6), evaluating for abnormal vertebrae morphology or number, and obtaining additional imaging to assess complications of the associated
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syndrome. Standing posterior-anterior radiograph should be obtained and assessed
for two pedicles at every level, extent of deformity in the coronal plane, and severity of rotation. If no rotational deformity is present, additional evaluation for spinal masses, syrinx, or bony tumors should be performed. Spinal magnetic
resonance imaging is also indicated in young patients under 10years of age, those
with neurologic decits, and in patients who present with spinal dysraphism. In
patients with suspected Marfan, obtain a cardiac echocardiogram. For patients
with Marfan, biconcave vertebrae, transitional vertebrae, and increased transverse
process distance are common, in addition to smaller pedicle widths and decreased
laminar thickness.
Cervical spine instability must be ruled out in patients with Trisomy 21; in
particular, atlantoaxial instability affects 10% to 20% of individuals with Down
syndrome and it is mostly asymptomatic; the condition can be diagnosed on plain
radiographs by an enlarged anterior atlanto-odontoid distance. Symptomatic
atlantoaxial instability (about 2% of cases) manifests with spinal cord compression (Chap. 3).
K. V. Suresh and P. D. Sponseller
14.6 Treatment Options
First-line treatment for syndromic scoliosis depends on the severity of the curve
upon initial patient presentation. On presentation, Cobb angle >20° (Video 14.4),
rib-vertebral angle difference (RVAD) >20°, and rib-vertebral overlap are predictors
of curve progression. In infants, an RVAD greater than 20° or a curve greater than
35° warrants Mehta casting (Video 14.5). If the curve continues to progress to
approximately 60° to 70° despite casting, consider growth rod placement. Similarly,
in older children, curves of 25° or greater are managed initially with bracing, with
denitive growth rod placement if the curve continues to progress to 50° to 60°
(Videos 14.1, 14.3, 14.5 and 14.7). In syndromic scoliosis patients with thoracic
insufciency syndrome due to signicant curvature, vertical expandable prosthetic
titanium rib (VEPTR) can also be considered to aid lung development, till denitive
growth rod placement or fusion can be performed.
14.7 Expected Outcomes
Syndromic scoliosis, compared to idiopathic scoliosis, is less likely to respond to
bracing and casting measures. Surgical intervention in these patients is associated
with greater risk of deep surgical infections, longer length of hospital admission,
and increased risk of pulmonary embolism and neurologic injury. Device-related
complications including failure and rates of pseudarthrosis are signicantly higher
in syndromic scoliosis patients.
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14.8 What Should Patient andFamily Know?
Syndromic scoliosis is a type of scoliosis that is secondary to an underlying disease
process. The presentation of syndromic scoliosis is highly variable. Scoliosis in
these patients starts earlier, progresses more rapidly, generally does not respond
well to bracing, and has a higher risk of complications with surgery.
Further Readings
Chung AS, Renfree S, Lockwood DB, Karlen J, Belthur M. Syndromic scoliosis: national
trends in surgical management and inpatient hospital outcomes: a 12-year analysis. Spine.
2019;44(22):1564–70. https://doi.org/10.1097/BRS.0000000000003134.
Janicki JA, Alman B. Scoliosis: review of diagnosis and treatment. Paediatr Child Health.
2007;12(9):771–6. https://doi.org/10.1093/pch/12.9.771.
Sullivan BT, Abousamra O, Puvanesarajah V, Jain A, Hadad MJ, Milstone AM, Sponseller PD.Deep
infections after pediatric spinal arthrodesis: differences exist with idiopathic, neuromuscular,
or genetic and syndromic cause of deformity. J Bone Joint Surg Am. 2019;101(24):2219–25.
Early Onset Neuromuscular Scoliosis
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KareemKebaish andPaulD.Sponseller
15.1 Definition
Early-onset scoliosis is associated with a variety of neuromuscular disorders. In
comparison to other forms of scoliosis, neuromuscular scoliosis often has an early
onset with rapid progression during growth, which may continue to progress following skeletal maturity. It is more likely to present with severe spinal deformities
that frequently involve the sacrum, and may have associated pelvic obliquity; respiratory compromise is frequent. The associated disorders are broadly classied as
either neuropathic or myopathic (Chap. 20).
15.2 Natural History
Progression throughout life is common as well as a respiratory compromise;
depending on the underlying disorder, progression can be extremely rapid.
Supplementary Information The online version contains supplementary material available at
[https://doi.org/10.1007/978- 3- 030- 80356- 8_15].
K. Kebaish
Department of Orthopaedic Surgery, Yale School of Medicine, New Haven, CT, USA
e-mail: kareem.kebaish@yale.edu
P. D. Sponseller (*)
Department of Orthopaedic Surgery, The Johns Hopkins University Hospital,
Baltimore, MD, USA
e-mail: psponse@jhmi.edu
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spine Surgery,
https://doi.org/10.1007/978-3-030-80356-8_15
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K. Kebaish and P. D. Sponseller
a
Fig. 15.1 A four-year-old patient with spinal muscular atrophy Type 1 with severe progressive
kyphoscoliosis (a and b), treated with MCGR (c and d)
b
c
d
15.3 Physical Examination
Neuropathic disorders, including cerebral palsy (CP), spinal muscular atrophy
(SMA), myelomeningocele, and Rett syndrome, are more commonly associated
with early onset (Videos 15.4 and 15.5). In contrast, some patients with myopathic
disorders such as Duchenne’s muscular dystrophy are most likely to develop scoliosis after the age of 10years old. Curve progression in all may continue after skeletal
maturity, and a respiratory compromise is frequent. Patients with SMA are notable
in that age of scoliosis onset may occur as early as less than 2years of age (Fig.15.1).
Earlier onset is predictive of more severe development of scoliosis. Patients with
myelomeningocele may develop tethered cord syndrome following surgical repair,
which may be a cause of progressive scoliosis. Management of these patients is
complicated by associated disorders including renal anomalies, and neurogenic
bowel and bladder. Successful management of patients with myelomeningoceles
requires a multi-disciplinary approach. Patients with Rett syndrome who present for
management will be female, as the X-linked mutation leads to death in males within
the rst 2years of life. As with other causes of neuromuscular scoliosis, patients
with Rett syndrome can develop rapidly progressive scoliosis, which may continue
after skeletal maturity.
15.4 Imaging
Radiographic evaluation of scoliosis involves determining the severity of the curve
(Video 15.6), evaluating for abnormal vertebrae morphology or number, and obtaining additional imaging to assess complications of the associated syndrome. Spinal
magnetic resonance imaging is also indicated in young patients under 10years of age,
those with neurologic decits, and in patients who present with spinal dysraphism.
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15.5 Treatment Options
Nonoperative management. Several goals of care, including maintenance of function and delay of surgical intervention (e.g., casting; Video 15.5), can be assisted by
non-operative interventions. Bracing can be used to control the progression of
curves during growth periods and delay the need for surgery until it can be performed safely. Patients with pulmonary conditions should be closely monitored, as
bracing can cause decreased chest wall expansion. Wheelchair modications, such
as trunk support and posterior contouring, allow patients to sit in a functional position and maintain more independence. Frequent skin evaluations in patients treated
with bracing or wheelchair modication are important in preventing the development of skin breakdown and pressure ulcers.
Intrathecal Nusinersen injections have improved the prognosis of SMA Type 1
and 2 patients. The medication is delivered directly to the central nervous system,
which allows it to be distributed to the spinal motor neurons located in the spinal cord.
Surgery. Surgical intervention may be considered in patients with rigid curves
greater than ~60° to 70°. A curve may be considered rigid when it has <50% of
exibility. Similar to conservative management, the goals of surgical intervention
are to control curve progression and provide stability in the coronal and sagittal
plane. Benets include decreased pain, improved pulmonary function, improved
seat positioning, and increased functional independence. Surgical options can be
broadly categorized into denitive fusion and growth-friendly non-fusion techniques. In DMD patients cardiac function is an important parameter to consider
when surgery is planned; anticipation is the best strategy.
Compared to idiopathic and congenital scoliosis, risks of surgery are greater in
patients with neuromuscular scoliosis as a result of their underlying disorders. A
preoperative assessment including a detailed history and evaluation of cardiac,
respiratory, neurological, and urologic function should be performed. Furthermore,
nutritional status and possible concomitant metabolic bone disease should be
assessed.
The use of non-fusion surgical techniques has become favored in the treatment
of early-onset scoliosis before a skeletal age of about 9years as, compared to fusion,
non-fusion techniques allow for the continued spine and chest growth. In addition
to traditional growing rods, many non-fusion options are available, including magnetically controlled growing rods (MCGR), vertical expandable prosthetic titanium
rib (VEPTR), Shilla growth guidance, self-expandable domino mechanical growing
rods, and localized fusion in myelomeningocele (Videos 15.3 and 15.7). Compared
to traditional growing rods, MCGR and the Shilla technique reduce the need for
multiple operations to expand the construct as the patient grows. VEPTR constructs
may be helpful in improving pulmonary function but require surgical adjustment
every 6–8months, increasing the risk of complications.
The extent of pelvic obliquity should be assessed in patients with neuromuscular
scoliosis. Pelvic obliquity can cause uneven weight distribution while patients are
sitting, leading to pressure ulcers and decreased sitting tolerance due to pain. Pelvic
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K. Kebaish and P. D. Sponseller
xation may be performed with fusion or as a part of a growing rod construct. The
typical criterion for pelvic xation is pelvic obliquity >10° to 15°, as measured on
the anteroposterior radiograph.
Denitive surgical fusion is commonly viewed as the endpoint of treatment for
neuromuscular scoliosis. Nevertheless, with the increased use of growth-friendly
techniques, denitive instrumentation and fusion may not be necessary for all
patients (Videos 15.1 and 15.5). Many patients treated with growth-friendly techniques undergo varying degrees of autofusion. Thus, patients with evidence of autofusion, minimal gain in length during their nal distraction, and no implant-related
complications can potentially be observed rather than undergo denitive fusion.
15.6 Expected Outcomes
Neuromuscular scoliosis, compared to idiopathic scoliosis, is less likely to respond
to bracing and casting measures. Surgical intervention in these patients is associated
with a greater risk of deep surgical infections, a longer length of hospital admission,
and increased risk of pulmonary embolism and neurologic injury.
In comparison to idiopathic and congenital scoliosis patients, patients with neuromuscular scoliosis have high rates of complications associated with both treatment and their underlying disease state. Neuromuscular scoliosis patients, especially
those with CP, have high rates of infection. Furthermore, patients may experience
pulmonary insufciency due to severe scoliosis curvature or associated deformities
such as kyphosis. Uneven sitting posture and treatment with bracing increase risks
of skin breakdown and pressure ulcers. Poor nutritional status in these patients is
associated with decreased immune function, increasing the risk of surgical complications such as wound infections and sepsis. A goal serum albumin of >3.5mg/dL
should be reached prior to surgical intervention. Patients treated with growing rods
that necessitate repeated distraction may experience progressive stiffness. In addition, instrumentation failure such as rod breakage or loosening of implants may
occur. Finally, some curves may rapidly progress despite attempts to control them
with growth-friendly techniques, leading to a more severe deformity that necessitates denitive surgical correction and fusion.
Further Readings
Allam AM, Schwabe AL.Neuromuscular scoliosis. PM R. 2013;5(11):957–63.
Jain A, Sponseller PD, Flynn JM, Shah SA, Thompson GH, Emans JB, Pawelek JB, Akbarnia
BA.Avoidance of “nal” surgical fusion after growing-rod treatment for early-onset scoliosis.
JBJS. 2016;98(13):1073–8.
McCarthy RE.Management of neuromuscular scoliosis. Orthop Clin N Am. 1999;30(3):435–49.
Idiopathic Scoliosis
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JeanDubousset
Two important points should be remembered when dealing with patients with idiopathic scoliosis:
1. Stiff deformity, painful deformity, or abnormal abdominal reexes are not sug-
gestive of idiopathic scoliosis.
2. Maximum reduction of the deformity by surgery is not always the optimal for
balance, harmony, and function.
16.1 Definition
Idiopathic scoliosis (IS) is a deformity of the axial skeleton that develops from head
to pelvis (Videos 16.6 and 16.10); IS is in relation with the erect posture of the
human and it is a three-dimensional deformity located in the sagittal, coronal, and
horizontal (rotation) plane [1]; the torsion of the vertebral column is the key deformity. It is called idiopathic because until now no clear etiology has been established.
Most advanced research converges to a genetic neuro-hormonal cause in relation to
the erect posture in otherwise healthy individuals.
IS involves up to 3% of the population (>90% females), and it must be distinguished from a scoliotic attitude where torsion does not exist.
Supplementary Information The online version contains supplementary material available at
[https://doi.org/10.1007/978- 3- 030- 80356- 8_16].
J. Dubousset (*)
French National Academy of Medicine, Paris, France
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spine Surgery,
https://doi.org/10.1007/978-3-030-80356-8_16
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J. Dubousset
16.2 Natural History
IS can be articially classied according to age into infantile (0–3years) (Chap.
13), juvenile (3–9 years), adolescent (9–end of growth) (Chap. 17) and adult
(neglected case or de novo deformity) (Chaps. 48 and 49). The majority of IS cases
show typical deformity progression (worsening of the torsion) during the adolescent
growth spurt (Appendix M). The rate and severity of deformity progression during
childhood and adolescence is extremely variable. Generally, the end of skeletal
growth stabilizes the deformity even though the deformity tends to progress mainly
at lumbar level during adulthood.
The three-dimensional architecture of the deformity [2] and the anatomical location of the deformity are responsible of the major related problems:
Thoracic spine: Apart from the cosmetic issue of the hump, the respiratory problems are predominant if signicant coronal or sagittal deformity exists.
Thoraco-lumbar and lumbar spine: cosmetic issues (waist asymmetry) and balance problems are predominant; moreover, the rotatory disorders (degenerative)
become predominant during adulthood and are responsible for pain (Chap. 45).
Physical Examination (1) Patients do not complain of pain nor stiffness. A painful and stiff scoliosis is from tumor, trauma, or infection until proven the contrary.
(2) The patient is clinically checked in a standing posture (Fig.16.1a): (a) to rule out
lower limb discrepancy; (b) the plumb line dropped from C7 help to detect and
a
Fig. 16.1 Clinical view of a typical adolescent idiopathic patient. She has an apparent truncal
shift and waistline asymmetry (a). Adam’s forward bending test of the patient shows a right rib
hump (gibbosity) which represents the axial rotation of vertebrae at the thoracic spine (b).
(Courtesy of Prof. Jean Dubousset)
b
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quantify right/left imbalance; (c) the Adam’s forward bending test is used to detect,
locate and measure the gibbosity (with the aid of the “scoliometer”) (Fig.16.1b); (d)
to detect and record the severity of waist asymmetry (window sign); (e) additional
signs: shoulder balance (elevated/symmetric/asymmetric), sagittal plane abnormality (kyphosis/lordosis; anterior hump. (3) Inspect the skin of the entire body to
detect “café au lait” spots suggestive of Neurobromatosis type-1 or McCuneAlbright syndrome; search for angioma (midline or a tuft hair predicting spinal cord
anomaly (Chap. 31). It is wise to check the elasticity of the skin and of the joints of
the hand to rule out connective tissue diseases such as Ehlers Danlos, Marfan; if
such diagnosis is suspected, echocardiography is more important than the radiographs of the spine in order to detect a latent aneurysm of the aorta! Complete
neurological examination is mandatory: the examiner must check the cranial
nerves, both upper and lower extremity osteo-tendinous reexes, spasticity and clonus, Babinski, and extrapyramidal function (Videos 16.4 and 16.9); abdominal
reexes must always be texted as the absence of cutaneous abdominal reex may
lead to suspecting a spinal dysraphism (Chiari malformation, syringomyelia)
(Chaps. 30 and 31).
Respiratory and pulmonary function (pulmonary function tests) must be
assessed at rest and during effort. It is important to remember that growth of the
spine and growth of the lungs are linked together from birth to 7years of age when
alveolar multiplication stops.
A precise evaluation of secondary sexual characteristics is needed though it
requires a lot of tact; breast and pubic hair development in girls as well as knowledge about the menarche status: growth spurt starts with the rst pubic hairs and
stops with the full horizontal pubic hair development; the rst menarche is the end
of the ascending phase of the pubertal diagram (Appendix M). In boys the augmentation of testis size highlights the beginning of the pubertal growth spurt which ends
with the appearance of the facial hair (beard).
Finally, it is wise to check the family history and to rule out if other family mem-
bers have the disease because of the frequent genetic inheritance.
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16.3 Imaging
Remember radiographs are only the shadows of the three-dimensional reality as
they can show the spine in two planes only. The essential imaging is the full spine
standing (anterior-posterior and lateral projections); the cervical spine up to the
skull and the entire pelvis should be included (if possible with low dose radiation
allowing a 3D computer reconstruction of the spine; EOS system). The full spine
radiographs allow locating and measuring all the curves (Video 16.6) and identifying the apex of the deformity, the “junction” between curves and between spinal
segments and the axial rotation of each vertebra. The Cobb angle with its perfect
reproducibility is still the basic measurement; the evaluation of the apex and of the
junctional levels must be done on both projections.
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