Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6012_Библиотеки_им_академика_М_И_Перельмана.pdf
AB
CD
Chapter 27 Idiopathic Scoliosis 463
SECTION
IV
E F GH
FIG. 27.8 (A–D) Radiographs of a girl, age 12 years + 6 months, with a severe progressive right thoracic
scoliosis. Her curve progressed to 159 degrees bending to only 135 degrees. (E–F) She underwent a posterior
single-level vertebrectomy and T2–L4 fusion with pedicle screw construct with marked correction of her
coronal plane deformity at 5 years postoperatively. (G–H) Preoperative and postoperative clinical photographs
show her much improved clinical appearance.
releases, leading to an unbalanced spine, account for other
types of intraoperative complications. Inappropriate decortication, inadequate bone gra material, and the use of bulky
crosslinks can result in a higher rate of pseudarthrosis.
Intraoperative neurophysiologic monitoring with somatosensory evoked potentials, motor evoked potentials, and/or
descending neurogenic evoked potentials help alert the
surgeon to any impending intraoperative spinal cord neurologic decit.
115,116
ese decits typically occur from spinal
cord distraction, overcorrection, vascular compromise, or,
rarely, directly from instrumentation. If intraoperative neurophysiologic monitoring declines past warning criteria, the
surgeon should implement a course of action that includes
ensuring that the irrigation being used is of adequate temperature, keeping mean arterial blood pressure elevated at a
minimum greater than 80 to 90 mm Hg, and reversing instrumentation or spinal correction to the prewarning criteria state.
If intraoperative neurophysiologic monitoring data do not
return to baseline within a reasonable time, a wake-up test
should also be performed to assess true neurologic function.
In addition to adhering to the proven sequential technique of
freehand screw placement, pedicle screw stimulation provides
an added safety measure.
79,80
Judicious use of intraoperative
imaging can also be employed, especially with signicant
deformities.
Postoperative complications can arise from delayed consequences of technical errors, neurovascular compromise,
medical comorbidities, and wound infections. Although
perioperative antibiotics are commonly used, when wound
infections do occur, they generally are treated aggressively
with wound irrigation and debridement. Instrumentation
well seated on the spine is always le in place; however, the
decision to remove or maintain the bone gra is dened by
the individual case and surgeon preference. Removal of the
464 PEDIATRICS
instrumentation can lead to loss of curve correction and
decompensation.
117
Additionally, at nal closure, powder
antibiotics can be placed inside the wound (deep and supercial to the fascia), and long-term parenteral antibiotics
are provided based on the results of intraoperative wound
cultures and sensitivities. With delayed or late infections, the
instrumentation is initially removed and later usually replaced
because the deformity can progress as the fusion mass is
subject to repeated bending forces.
117,118
Also, the fusion mass
is inspected further, and any pseudarthrosis noted is repaired
at the reinstrumentation stage.
Summary
Understanding and treatment of spinal deformities has broadened; however, idiopathic scoliosis remains a diagnosis of
exclusion. With advances in genetic mapping of idiopathic
scoliosis, better understanding of the etiology and incidence
of the disease is promising. It is hoped that better understanding will bring earlier identication, more insight into curve
progression risk, and treatments of the condition without
the need for major surgery of severe curves. Technological
advances—including the advent of pedicle screw instrumentation, better derotation equipment, and osteotomy techniques—
have signicantly improved spinal surgeons’ ability to treat
more rigid curves while obtaining better correction and
maintaining safety.
Possible treatment modalities include close observation,
bracing, and surgical intervention. e Lenke classication of
AIS allows for the identication of appropriate fusion levels and
choice of selective fusions, which are imperative for optimal
surgical management. Although all curves can be approached
posteriorly, one can employ an anterior approach in selected
Lenke 1 curve patterns, Lenke 6CN curves, and many Lenke
5CN curves. Selective fusions should be performed whenever
possible, and critical curve analysis should be performed
preoperatively with all available objective modalities. Direct
vertebral rotation oers improved thoracic correction and a
decreased need for thoracoplasty. Complex, decompensated,
large, rigid curves and curves previously fused may require
osteotomies to achieve the desired correction. Some pitfalls
of scoliosis surgery, such as decompensation and adding on
of a fused curve, can be avoided when these principles are
applied.
One must be mindful of the lessons of the past in understanding the assessment and management of spinal deformity.
Spinal surgeons constantly must strive for improvements in
surgical technique that lead to shorter, selective fusions and a
balanced spine with maximum possible correction. ese
improvements can include less invasive approaches while not
forgetting the basic principles of curve identication and
fusion techniques. e ultimate goal should be correction of
the curve without fusion of unnecessary vertebrae, allowing
for continued spinal motion. Safety for patients is of the
utmost importance and is achieved by appropriate training,
careful patient selection, and adherence to the principles of
deformity surgery.
PEARLS AND PITFALLS
Bracing of the juvenile idiopathic or skeletally immature
1.
adolescent idiopathic patient is still a viable option for those
with curves between 25 degrees and 45 degrees. Brace
compliance, the t of the orthosis, and the number of hours of
brace wear per day are critical components to success, along
with the genetic predisposition toward curve progression.
2.
It is important to determine the exibility of the spinal deformity.
Therefore, preoperative radiographic assessment should include
not only upright radiographs, but also side-bending, supine,
push-prone, traction (if applicable), and hyperextension (for
hyperkyphosis) radiographs, alone or in combination.
3.
Proper classication of AIS curves preoperatively will aid in the
regions of the spine to be fused. One must remember to
include the thoracolumbar sagittal prole in preoperative
planning to prevent misclassication and incorrect surgical
management.
4.
It is critical to examine shoulder symmetry clinically and
radiographically, the PT curve (stiness and sagittal prole),
and degree of MT curve correction when selecting proximal
fusion levels in idiopathic scoliosis patients to obtain optimal
shoulder balance after surgery.
5.
Distal fusion levels are determined by the relationship among
the end, neutral, and stable vertebrae of the distal structural
curve to be fused, along with the TV in relation to the position
of those vertebrae to the CSVL. Most commonly, the distal
fusion level will be one level above stable if that level is fairly
neutral and the vertebra is at least “touched” by the CSVL on
the upright coronal lm (the TV rule), does not have much
rotation, and the disc below is parallel or closed on the
convexity.
6.
Satisfactory clinical and radiographic results can be achieved
with selective thoracic fusions of properly selected lumbar C
modier curves.
7.
The use of anterior procedures and circumferential surgery for
AIS has markedly decreased since 2000, with the predominant
use of posterior-only surgery with spinal osteotomies as
needed for adequate three-dimensional correction.
Disadvantages such as chest cage disruption (including
suboptimal pulmonary function), risk of implants abutting the
major vessels, and the ability to treat only a single curve at a
time have limited these approaches over time.
8.
The use of posterior instrumentation and fusion with or without
various forms of spinal osteotomies has become the mainstay
for the surgical management of pediatric and adult idiopathic
scoliosis deformities. All curve patterns can be managed by
surgeons familiar with the classic midline posterior approach.
9.
Surgical outcomes are based on radiographic parameters and
clinical assessments, such as scoliometer measurements and
shoulder height, as well as patient-reported outcome
questionnaires.
10.
The use of segmental pedicle screw xation for the posterior
treatment of pediatric and adult idiopathic scoliosis curves has
become the primary instrumentation construct. In addition,
thorough bone grafting with a combination of autogenous
bone, allograft bone, and/or the use of osteobiologics,
especially in the adult population, has become routine at many
centers throughout North America.
11.
Optimal surgical outcomes in the treatment of idiopathic
scoliosis deformities include proper patient selection, exacting
surgical technique, and a well-balanced spinal alignment with
minimal to no complications.
Chapter 27 Idiopathic Scoliosis 465
KEY POINTS
1. Patient evaluation skills and highly specialized technical skills are
essential for the scoliosis surgeon.
2.
Anterior approaches are possible, but all curves can be
addressed posteriorly.
3.
Selective fusions should always be considered when
appropriate.
4.
Adjuncts to posterior correction possibly can help obviate more
extensive approaches.
5.
Be careful in choosing appropriate fusion levels and leaving an
appropriate tilt on the LIV when performing a selective fusion.
6.
Avoidance and treatment of complications in the preoperative,
intraoperative, and postoperative periods is important.
7.
Some form of spinal cord monitoring is mandatory for all
scoliosis corrective procedures.
KEY REFERENCES
1. Lenke LG, Betz RR, Bridwell KH, et al. Intraobserver and
interobserver reliability of the classication of thoracic
adolescent idiopathic scoliosis. J Bone Joint Surg Am. 1998;80:
1097-1106.
This study showed poor to fair reliability of the King classication
of adolescent idiopathic scoliosis, questioning its usefulness as an
accurate system.
2.
Lenke LG, Betz RR, Harms J, et al. Adolescent idiopathic scoliosis:
a new classication to determine extent of spinal arthrodesis.
J Bone Joint Surg Am. 2001;83:1169-1181.
This new two-dimensional treatment-based AIS classication
system was found to have good to excellent reliability and allows
classication of all adolescent idiopathic scoliosis curves.
3.
Lenke LG, Betz RR, Clements D, et al. Curve prevalence of a
new classication of operative adolescent idiopathic scoliosis:
does classication correlate with treatment? Spine. 2002;27:
604-611.
Of 606 consecutive AIS cases classied by the Lenke et al system,
type 1 main thoracic curves were the most common (51%), and 90%
of curves were fused as predicted by the system.
4.
Sanders AE, Baumann R, Brown H, et al. Selective anterior fusion
of thoracolumbar/lumbar curves in adolescents: When can the
associated thoracic curve be left unfused? Spine.
2003;28:706-713.
Of 49 patients with AIS who underwent an anterior selective
thoracic fusion, 43 had satisfactory results based on the preoperative
thoracolumbar/lumbar-to-thoracic ratio of 1.25 or greater.
5.
Edwards CC II, Lenke LG, Peelle M, et al. Selective thoracic fusion
for adolescent idiopathic scoliosis with C modier lumbar
curves: 2- to 16- year radiographic and clinical results. Spine.
2004;29:536-546.
Satisfactory results were achieved with selective thoracic fusion of
properly selected C modier lumbar curves with under-correction of
the instrumented thoracic curve (36%) to match the spontaneous
correction of the lumbar curve (34%).
REFERENCES
1. Bunnell WP. e natural history of idiopathic scoliosis before
skeletal maturity. Spine. 1986;1:773-776.
2. Cobb JR. Outline for the study of scoliosis. Instr Course Lect.
1948;5:261-275.
3. Ponseti IV, Friedman B. Prognosis in idiopathic scoliosis.
J Bone Joint Surg Am. 1950;32:381-395.
4. Dickson RA. Conservative treatment for idiopathic scoliosis.
J Bone Joint Surg Br. 1985;67:176-181.
5. James JI. Idiopathic scoliosis: the prognosis, diagnosis, and
operative indications related to curve patterns and the age at
onset. J Bone Joint Surg Br. 1954;36:36-49.
6. Fernandes P, Weinstein SL. Natural history of early onset
scoliosis. J Bone Joint Surg Am. 2007;89(suppl 1):21-33.
7. Kane WJ, Moe JH. A scoliosis-prevalence survey in
Minnesota. Clin Orthop Relat Res. 1970;69:216-218.
8. Pring ME, Wenger DR. Adolescent deformity. In: Bono
CM, Garn SR, eds. Spine Orthopedic Surgery Essentials.
Philadelphia: Lippincott Williams & Wilkins; 2004:163-164.
9. Moreau A, Wang DS, Forget S, et al. Melatonin signaling
dysfunction in adolescent idiopathic scoliosis. Spine. 2004;29:
1772-1781.
10. Azeddine B, Letellier K, Wang da S, et al. Molecular
determinants of melatonin signaling dysfunction in
adolescent idiopathic scoliosis. Clin Orthop Relat Res. 2007;
462:45-52.
11. Yamamoto H. A postural dysequilibrium as an etiological factor
in idiopathic scoliosis [abstract O]. In Programs and Abstracts
of the 17th Annual Meeting of the Scoliosis Research Society,
Denver, 1982, p 52.
12. Machida M, Dubousset J, Imamura Y, et al. An experimental
study in chickens for the pathogenesis of idiopathic scoliosis.
Spine. 1993;18:1609-1615.
13. De Seze M, Cugy E. Pathogenesis of idiopathic scoliosis: a
review. Ann Phys Rehabil Med. 2012;55(2):128-138.
14. Kouwenhoven JW, Castelein RM. e pathogenesis of
adolescent idiopathic scoliosis: review of the literature. Spine.
2008;33:2989-2998.
15. Kulkarni S, Nagarajan P, Wall J, et al. Disruption of
chromodomain helicase DNA binding protein 2 (CHD2)
causes scoliosis. Am J Med Genet. 2008;146A:1117-1127.
16. Gao X, Gordon D, Zhang D, et al. CHD7 gene
polymorphisms are associated with susceptibility to idiopathic
scoliosis. Am J Hum Genet. 2007;80:957-965.
17. Tilley MK, Justice CM, Swindle K, et al. CHD gene
polymorphisms and familial idiopathic scoliosis. Spine.
2013;32(22):E1432-E1436.
18. Takahashi Y, Kou I, Takahashi A, et al. A genome-wide
association study identies common variants near LBX1
associated with adolescent idiopathic scoliosis. Nat Genet.
2011;43:1237-1240.
19. Hua J, Xusheng Q, Jin D, et al. Association of rs11190870 near
LBX1 with adolescent idiopathic Scoliosis susceptibility in a
Han Chinese population. Eur Spine J. 2013;22(2):282-286.
20. Risser JC, Norquist DM, Cockrell BR Jr, et al. e eect of
posterior spine fusion on the growing spine. Clin Orthop Relat
Res. 1966;46:127-139.
21. Carr AJ. Adolescent idiopathic scoliosis in identical twins.
J Bone Joint Surg Br. 1990;72:1077.
22. Kesling LK, Reinker KA. Scoliosis in twins: a meta-analysis
of the literature and report of six cases. Spine. 1997;22:
2009-2014.
23. Anderson MO, omsen K, Kyvik KO. Adolescent idiopathic
scoliosis in twins: a population-based survey. Spine.
2007;32:927-930.
24. Gurnett CA, Alaee F, Bowcock A, et al. Genetic linkage
localizes an adolescent idiopathic scoliosis and pectus
excavatum gene to 18q. Spine. 2009;4:E94-E100.
25. Diedrich O, von Strempel A, Scholz M, et al. Long-term
observation and management of resolving infantile
idiopathic scoliosis: a 25-year follow-up. J Bone Joint Surg Br.
2002;84:1030-1035.
SECTION
IV
466 PEDIATRICS
26. Mehta MH. e rib-vertebra angle in the early diagnosis
between resolving and progressive infantile scoliosis. J Bone
Joint Surg Br. 1972;54:230-243.
27. Weinstein SL, Ponseti IV. Curve progression in idiopathic
scoliosis. J Bone Joint Surg Am. 1983;65:447-455.
28. Weinstein SL, Zavala DC, Ponseti IV. Idiopathic scoliosis:
long-term follow-up and prognosis in untreated patients.
J Bone Joint Surg Am. 1981;63:702-712.
29. Hägg U, Taranger J. Menarche and voice change as
indicators of the pubertal growth spurt. Acta Odontol Scand.
1980;38(3):179-186.
30. Lonstein JE. Patient evaluation. In: Bradford DS, Lonstein
JE, Moe JH, et al., eds. Moe’s Textbook of Scoliosis and Other
Spinal Deformities. 2nd ed. Philadelphia: WB Saunders;
1987:47-88.
31. Li M, Gu S, Ni J, et al. Shoulder balance aer surgery in
patients with Lenke type 2 scoliosis corrected with the
segmental pedicle screw technique. J Neurosurg Spine.
2009;10:214-219.
32. Winter RB, Pinto WC. Pelvic obliquity: its causes and
treatment. Spine. 1986;11(3):225-234.
33. Muhonen MG, Menezes AH, Sawin PD, et al. Scoliosis in
pediatric Chiari malformations without myelodysplasia.
J Neurosurg. 1992;77:69-77.
34. Dobbs MB, Lenke LG, Szymanski DA, et al. Prevalence of
neural axis abnormalities in patients with infantile scoliosis.
J Bone Joint Surg Am. 2002;84:2230-2234.
35. Gupta P, Lenke LG, Bridwell KH. Incidence of neural
axis abnormalities in infantile and juvenile patients with
spinal deformity: Is a magnetic resonance image screening
necessary? Spine. 1998;23:206-210.
36. Whitaker C, Schoenecker PL, Lenke LG. Hyperkyphosis as
an indicator of syringomyelia in idiopathic scoliosis. Spine.
2003;28(1):E16-E20.
37. Godzik J, et al. Relationship of syrinx size and tonsillar
descent to spinal deformity in Chiari malformation type
I with associated syringomyelia. J Neurosurg Pediatr.
2014;13:368-374.
38. Williams BA, Matsumoto H, McCalla DJ, et al. Development
and initial validation of the classication of early-onset scoliosis
(C-EOS). J Bone Joint Surg Am. 2014;96(16):1359-1367.
39. Lenke LG, Dobbs MB. Management of juvenile idiopathic
scoliosis. J Bone Joint Surg Am. 2007;89:55-63.
40. King HA, Moe JH, Bradford DS, et al. e selection of fusion
levels in thoracic idiopathic scoliosis. J Bone Joint Surg Am.
1983;65:1302-1313.
41. Lenke LG, Bridwell KH, Baldus C, et al. Preventing
decompensation in King type II curves treated with
Cotrel-Dubousset instrumentation: strict guidelines for
selective thoracic fusion. Spine. 1992;17:274S-281S.
42. Schroeder TM, Blanke KM, Vaughan V, et al. Validation
of radiographic soware to determine Lenke classication
[abstract 54]. In Programs and Abstracts of the 40th Annual
Meeting of the Scoliosis Research Society, Miami, 2005, p 93.
43. Lenke LG, Betz RR, Bridwell KH, et al. Intraobserver and
interobserver reliability of the classication of thoracic
adolescent idiopathic scoliosis. J Bone Joint Surg Am.
1998;80:1097-1106.
44. Lenke LG, Betz RR, Harms J, et al. Adolescent idiopathic
scoliosis: a new classication to determine extent of spinal
arthrodesis. J Bone Joint Surg Am. 2001;3:1169-1181.
45. Cil A, Pekmezci M, Yazici M, et al. e validity of Lenke’s
criteria for dening structural proximal thoracic curves in
patients with adolescent idiopathic scoliosis [abstract 74]. In
Programs and Abstracts of the 40th Annual Meeting of the
Scoliosis Research Society, Miami, 2005, p 120.
46. Sanders AE, Baumann R, Brown H, et al. Selective anterior
fusion of thoracolumbar/lumbar curves in adolescents: When
can the associated thoracic curve be le unfused? Spine.
2003;28:706-713.
47. Edwards CC 2nd, Lenke LG, Peelle M, et al. Selective thoracic
fusion for adolescent idiopathic scoliosis with C modier
lumbar curves: 2- to 16- year radiographic and clinical results.
Spine. 2004;29:536-546.
48. Lenke LG, Bridwell KH. Achieving coronal balance
using Cotrel-Dubousset instrumentation (C-D.I.). In
the 8th Proceedings of the International Congress on
Cotrel-Dubousset Instrumentation. Montpellier, Sauramps
Medical, 1991, pp 27-32.
49. Lenke LG, Kuklo TR, Sucato DJ, et al. Comparison of the
lower-end vertebra (LEV) to the lowest instrumented vertebra
(LIV) in adolescent idiopathic scoliosis: a role for the addition
of an LEV modier to the Lenke classication system [abstract
131]. In Programs and Abstracts of the 13th International
Meeting on Advanced Spine Techniques (IMAST), Athens,
2006, p 121.
50. Lenke LG, Newton PO, Lehman RA, et al. Radiographic results
of selecting the touched vertebra as the lowest instrumented
vertebra in Lenke 1A AIS curves at a minimum ve-year
follow up. Podium presentation 5. Scoliosis Research
Society 49
th
Annual Meeting, Anchorage, AK, September
11-13, 2014.
51. Sangole A, Aubin CE, Labelle H, et al. e central hip vertical
axis (CHVA): a reference axis for the Scoliosis Research
Society three-dimensional classication of idiopathic scoliosis.
Spine. 2010;35(12):E530-E534.
52. Nachemson AL, Peterson LE. Eectiveness of treatment with
a brace in girls who have adolescent idiopathic scoliosis: a
prospective, controlled study based on data from the Brace
Study of the Scoliosis Research Society. J Bone Joint Surg Am.
1995;77:815-822.
53. Stone B, Beekman C, Hall V, et al. e eect of an exercise
program on change in curve in adolescents with minimal
idiopathic scoliosis: a preliminary study. Phys er.
1979;59:759-763.
54. Romano M1, Minozzi S, Zaina F, et al. Exercises for
adolescent idiopathic scoliosis: a Cochrane systematic review.
Spine. 2013;38(14):E883-E889.
55. Mordecai SC, Dabke HV. Ecacy of exercise therapy for the
treatment of adolescent idiopathic scoliosis: a review of the
literature. Eur Spine J. 2012;21(3):382-389.
56. Weinstein SL. Idiopathic scoliosis: natural history. Spine.
1986;11:780-783.
57. Fayssoux RS, Cho RH, Herman MJ. A history of bracing for
idiopathic scoliosis in North America. Clin Orthop Relat Res.
2010;468:654-664.
58. Kennedy JD, Robertson CF, Olinsky A, et al. Pulmonary
restrictive eect of bracing in mild idiopathic scoliosis.
orax. 1987;42:959-961.
59. Katsaris G, Loukos A, Valavanis J, et al. e immediate
eect of a Boston brace on lung volumes and pulmonary
compliance in mild adolescent idiopathic scoliosis. Eur Spine
J. 1999;8:2-7.
60. Sanders JO, D’Astous J, Fitzgerald M, et al. Derotational
casting for progressive infantile scoliosis. J Pediatr Orthop.
2009;29:581-587.
Chapter 27 Idiopathic Scoliosis 467
61. Blount WP, Schmidt AC, Keever ED, et al. e Milwaukee
brace in the operative treatment of scoliosis. J Bone Joint Surg
Am. 1958;40:511-525.
62. Price CT, Scott DS, Reed FR Jr, et al. Nighttime bracing for
adolescent idiopathic scoliosis with the Charleston bending
brace: long-term follow-up. J Pediatr Orthop. 1997;17:703-707.
63. Trivedi JM, omson JD. Results of Charleston bracing
in skeletally immature patients with idiopathic scoliosis. J
Pediatr Orthop. 2001;21:277-280.
64. Clin J, Aubin CE, Parent S, et al. A biomechanical study of
the Charleston brace for the treatment of scoliosis. Spine.
2010;35:E940-E947.
65. Kotwicki T, Chêneau J. Biomechanical action of a correction
brace of thoracic idiopathic scoliosis: Chêneau 2000 orthosis.
Disabil Rehabil Assist Technol. 2008;3:146-153.
66. Rigo M, Negrini S, Weiss H, et al. SOSORT consensus
paper on brace action: TLSO biomechanics of correction
(investigating the rationale for force vector selection).
Scoliosis. 2006;1:11.
67. Szwed A, Kolban M, Jaloszewski M. Results of SpineCor
dynamic bracing for idiopathic scoliosis. Ortop Traumatol
Rehabil. 2009;11:427-432.
68. Maruyama T, Takeshita K. Surgical treatment of scoliosis: a
review of techniques currently applied. Scoliosis. 2008;18:6.
69. Dobbs MB, Weinstein SL. Infantile and juvenile scoliosis.
Orthop Clin North Am. 1999;30:331-341.
70. Lenke LG, Dobbs MB. Management of juvenile idiopathic
scoliosis. J Bone Joint Surg Am. 2007;89(suppl 1):55-63.
71. Marks DS, Iqbal MJ, ompson AG, et al. Convex spinal
epiphysiodesis in the management of progressive infantile
idiopathic scoliosis. Spine. 1996;21:1884-1888.
72. Bylski-Austrow DI, Wall EJ, Glos DL, et al. Spinal
hemiepiphysiodesis decreased the sizes of vertebral growth
plate hypertrophic zone and cells. J Bone Joint Surg Am.
2009;91:854-893.
73. Akbarnia BA, Marks DS, Boachie-Adjei O, et al. Dual
growing rod technique for the treatment of progressive
early-onset scoliosis: a multicenter study. Spine. 2005;30(17
suppl):S46-S57.
74. Betz RR, Kim J, D’Andrea LP, et al. An innovative technique
of vertebral body stapling for the treatment of patients with
adolescent idiopathic scoliosis: a feasibility, safety, and utility
study. Spine. 2003;28:S255-S265.
75. Betz RR, Ranade A, Samdani AF, et al. Vertebral body
stapling: a fusionless treatment option for a growing child
with moderate idiopathic scoliosis. Spine. 2010;35:169-176.
76. Newton PO, Upasani VV, Farnsworth CL, et al. Spinal growth
modulation with use of a tether in an immature porcine
model. J Bone Joint Surg Am. 2008;90:2695-2706.
77. Crawford CH III, Lenke LG. Growth modulation by means
of anterior tethering resulting in progressive correction of
juvenile idiopathic scoliosis: a case report. J Bone Joint Surg
Am. 2010;92:202-209.
78. ompson GH, Akbarnia BA, Campbell RM Jr. Growing
rod techniques in early-onset scoliosis. J Pediatr Orthop.
2007;27:354-361.
79. Lenke LG, Rinella A, Kim Y. Freehand thoracic pedicle screw
placement. Semin Spine Surg. 2002;14:48-57.
80. Kim YJ, Lenke LG, Bridwell KH, et al. Free hand pedicle
screw placement in the thoracic spine: Is it safe? Spine.
2004;29:333-341.
81. Good CR, Lenke LG, O’Leary PT, et al. Can posterior-only
surgery provide similar radiographic and clinical results
as combined anterior (thoracotomy/thoracoabdominal)/
posterior approaches for adult scoliosis? Spine.
2010;35:210-218.
82. Kioschos HC, Asher MA, Lark RG, et al. Overpowering
the cranksha mechanism: the eect of posterior spinal
fusion with and without sti transpedicular xation on
anterior spinal column growth in immature canines. Spine.
1996;21:1168-1173.
83. Burton DC, Asher MA, Lai SM. Scoliosis correction
maintenance in skeletally immature patients with idiopathic
scoliosis: Is anterior fusion really necessary? Spine.
2000;25:61-68.
84. Suk SI, Kim JH, Cho KJ, et al. Is anterior release necessary in
severe scoliosis treated by posterior segmental pedicle screw
xation? Eur Spine J. 2007;16:1359-1365.
85. Dobbs MB, Lenke LG, Kim YJ, et al. Anterior/posterior spinal
instrumentation versus posterior instrumentation alone for
the treatment of adolescent idiopathic scoliotic curves more
than 90°. Spine. 2006;31:2386-2391.
86. Kim YJ, Lenke LG, Bridwell KH, et al. Pulmonary function
in adolescent idiopathic scoliosis relative to the surgical
procedure. J Bone Joint Surg Am. 2005;87:1534-1541.
87. Lonner BS, Auerbach JD, Estreicher MB, et al. Pulmonary
function changes aer various anterior approaches in the
treatment of adolescent idiopathic scoliosis. J Spinal Disord
Tech . 2009;22:551-558.
88. Tis JE, O’Brien MF, Newton PO, et al. Adolescent idiopathic
scoliosis treated with open instrumented anterior spinal
fusion: ve-year follow-up. Spine. 2010;35:64-70.
89. Geck MJ, Rinella A, Hawthorne D, et al. Comparison of
surgical treatment in Lenke 5C adolescent idiopathic scoliosis:
anterior dual rod versus posterior pedicle xation surgery: a
comparison of two practices. Spine. 2009;34:1942-1951.
90. Kishan S, Bastrom T, Betz RR, et al. oracoscopic scoliosis
surgery aects pulmonary function less than thoracotomy at 2
years postsurgery. Spine. 2007;32:453-458.
91. Sucato DJ, Erken YH, Davis S, et al. Prone thoracoscopic
release does not adversely aect pulmonary function when
added to a posterior spinal fusion for severe deformity. Spine.
2009;34:771-778.
92. Bridwell KH, Lenke LG. Prevention and treatment of
decompensation: When can levels be saved and selective
fusion be performed in idiopathic scoliosis? Spine State Art
Rev. 1994;8:643-658.
93. Suk SI, Lee SM, Chung ER, et al. Selective thoracic fusion
with segmental pedicle screw xation in the treatment of
thoracic idiopathic scoliosis: more than 5-year follow-up.
Spine. 2005;30:1602-1609.
94. Brodner W, Mun Yue W, Möller HB, et al. Short segment
bone-on-bone instrumentation for single curve idiopathic
scoliosis. Spine. 2003;28:S224-S233.
95. O’Brien MF, Kuklo TR, Blanke KM, et al., eds. Radiographic
Measurement Manual. Memphis, TN: Medtronic Sofamor
Danek; 2004.
96. Rinella A, Cahill P, Ghanayem A, et al. oracic pedicle
expansion aer pedicle screw placement in a pediatric cadaveric
spine: A biomechanical analysis [abstract 35]. In Programs
and Abstracts of the 39th Annual Meeting of the Scoliosis
Research Society, Argentina, 2004, p 70.
97. Watanabe K, Lenke LG, Matsumoto M, et al. A novel pedicle
channel classication describing osseous anatomy: How many
thoracic scoliotic pedicles have cancellous channels? Spine.
2010;35(20):1836-1842.
SECTION
IV
468 PEDIATRICS
98. Bridwell KH, McAllister JW, Betz RR, et al. Coronal
decompensation produced by Cotrel-Dubousset “derotation”
maneuver for idiopathic right thoracic scoliosis. Spine.
1991;16:769-777.
99. Lehman RA Jr, Lenke LG, Helgeson MD, et al. Do
intraoperative radiographs in scoliosis surgery reect
radiographic result? Clin Orthop Relat Res. 2010;468:
679-686.
100. Donaldson S, Stephens D, Howard A, et al. Surgical
decision making in adolescent idiopathic scoliosis. Spine.
2007;32:1526-1532.
101. Keeler KA, Lehman RA, Lenke LG, et al. Direct vertebral
rotation (DVR) in the treatment of thoracolumbar/lumbar
adolescent idiopathic scoliosis (AIS): can it optimize correction
when fusing to L3? [abstract 137]. In Programs and Abstracts
of the 15th International Meeting on Advanced Spine
Techniques, Hong Kong, 2008, p 215.
102. Lee SM, Suk SI, Chung ER. Direct vertebral rotation: a new
technique of three-dimensional deformity correction with
segmental pedicle screw xation in adolescent idiopathic
scoliosis. Spine. 2004;29:343-349.
103. Kadoury S, Cheriet F, Beauséjour M, et al. A
three-dimensional retrospective analysis of the evolution
of spinal instrumentation for the correction of adolescent
idiopathic scoliosis. Eur Spine J. 2009;18:23-37.
104. Bridwell KH. Adult spinal deformity revision surgery. In:
Heary RF, Albert TJ, eds. Spinal Deformity: e Essentials.
New York: ieme; 2007:240-248.
105. Booth KC, Bridwell KH, Lenke LG, et al. Complications and
predictive factors for the successful treatment of atback
deformity (xed sagittal balance). Spine. 1999;24:
1712-1720.
106. Ondra SL, Marzouk S, Koski T, et al. Mathematical
calculation of pedicle subtraction osteotomy size to allow
precision correction of xed sagittal deformity. Spine.
2006;31:E973-E979.
107. Bradford DS, Tribus CB. Vertebral column resection for
the treatment of rigid coronal decompensation. Spine.
1997;22:1590-1599.
108. Lenke LG, O’Leary PT, Bridwell KH, et al. Posterior vertebral
column resection for severe pediatric deformity: minimum
two-year follow-up of thirty-ve consecutive patients. Spine.
2009;34:2213-2221.
109. Lenke LG, Sides BA, Koester LA, et al. Vertebral column
resection for the treatment of severe spinal deformity. Clin
Orthop Relat Res. 2010;468:687-699.
110. Lenke LG, Ogilvie JG, Mumamenni P, Silva FE. Safety
measures and operative planning in the treatment of spinal
deformities. Scoliosis Research Society, E-Text, 2013.
111. Newton PO, Marks M, Faro F, et al. Use of video-assisted
thoracoscopic surgery to reduce perioperative morbidity in
scoliosis surgery. Spine. 2003;28:S249-S254.
112. Lykissas MG, Crawford AH, Jain VV. Complications of
surgical treatment of pediatric spinal deformities. Orthop Clin
North Am. 2013;44(3):357-370.
113. Lenke LG, Bridwell KH, Erickson MA, et al. Prospective
radiographic and clinical outcomes and complications of 756
consecutive operative adolescent idiopathic scoliosis patients
[abstract 3]. In Programs and Abstracts of the 44th Annual
Meeting of the Scoliosis Research Society, San Antonio, TX,
2009, p 39.
114. Trobisch PD, Ducoe AR, Lonner BS, Errico TJ. Choosing
fusion levels in adolescent idiopathic scoliosis. J Am Acad
Orthop Surg. 2013;21(9):519-528.
115. Padberg AM, Wilson-Holden TJ, Lenke LG, et al.
Somatosensory- and motor-evoked potential monitoring
without a wake-up test during idiopathic scoliosis surgery: an
accepted standard of care. Spine. 1998;23:1392-1400.
116. Cho SK, Lenke LG, Bolon SM, et al. Can intraoperative spinal
cord monitoring reliably help prevent paraplegia during
posterior vertebral column resection surgery? Spine Deform.
2015;3:73-81.
117. Potter BK, Kirk KL, Shah SA, et al. Loss of coronal correction
following instrumentation removal in adolescent idiopathic
scoliosis. Spine. 2006;31:67-72.
118. Luhmann SJ, Lenke LG, Bridwell KH, et al. Revision surgery
aer primary spine fusion for idiopathic scoliosis. Spine.
2009;34:2191-2197.
SECTION
28
CHAPTER
General Principles
Neuromuscular disorders commonly lead to spinal deformities that are some of the most challenging treatment dilemmas
addressed by spine surgeons. Despite the various conditions
that fall in this category, neuromuscular disorders involve
neurologic or muscular deciencies that produce progressive
multiplanar skeletal deformities. Common features of neuromuscular scoliosis include the following:
Large curves early in life: Early neuromuscular insult pre-
disposes patients to rapidly progressive scoliosis.
Sti curves: ese patients are more likely to develop sti
curves because of the early onset of neuromuscular deciency,
resulting in limited mobility and secondary contractures.
Progressive curves: As in idiopathic scoliosis, the potential
for curve progression is greatest during rapid growth and with
loss of ambulation. Increasing weakness or persistent muscle
imbalance around the spine in patients with neuromuscular
disorders can cause progression of scoliosis independent of
growth, however.
Long curves: Less severely aected individuals may have an
S-shaped curve with well-balanced double curves. Long
C-shaped curves are more likely in severely aected patients
with resultant sitting imbalance.
Pelvic obliquity: Lower extremity contractures and imbalanced spinal deformity cause pelvic obliquity, which may
impair comfortable sitting for these patients.
Sagittal plane deformity: Gravity and muscular deciency
can also lead to sagittal plane deformity, including thoracic or
lumbar hyperkyphosis or lumbar hyperlordosis.
Patients with neuromuscular disorders are challenging
because of the complexity of their deformity and fragility of
their overall health; they are best treated by an experienced
surgeon with support from a multidisciplinary team.
Neuromuscular Scoliosis
Peter O. Newton
Pawel P. Jankowski
Burt Yaszay
Dennis R. Wenger
Scott J. Mubarak
muscular (e.g., muscular dystrophy). Neurologic deciencies
can be broken down further into upper motor neuron dysfunction, as seen in myelomeningocele, or lower motor neuron
dysfunction, as seen in spinal muscular atrophy (SMA).
Natural History and Associated Complications
Neuromuscular scoliosis generally begins early in life, is oen
rapidly progressive, and causes signicant morbidity. Some
patients are capable of ambulation, although many lose their
ability to walk early in life or never achieve ambulatory status
at all. e use of a wheelchair aords these patients educational
and social opportunities that enrich their lives. Spinal deformity can impair comfortable sitting and dramatically reduce
the individual’s quality of life. Unbalanced curves and signicant pelvic obliquity make wheelchair positioning dicult and
may cause uneven distribution of weight that may lead to
pressure sores (Fig. 28.1). Prominences created by the convexity of a curve may result in skin breakdown; creases within the
concavity of the trunk deformity are susceptible to skin
maceration and infection (Fig. 28.2). Majd and colleagues1
showed a correlation between deformity size, functional
decline, and decubitus. Large rigid curves restrict lung volume
and impair respiration in patients who oen already have
limited pulmonary capacity. Treatment of neuromuscular
scoliosis can also help the caretakers of these patients, improving the ease of transfers, positioning, feeding, and hygiene.
e ultimate goal of treatment of patients with neuromuscular
scoliosis is the maintenance of as much independence and
function as possible. When patients with neuromuscular
scoliosis lose the ability to sit comfortably, their quality of life
is dramatically decreased. e natural history for a given
patient is largely determined by the specic underlying neu-
romuscular condition and the degree of involvement.
IV
Classication
e classication of neuromuscular scoliosis can be based on
the underlying disorder: neurologic (e.g., cerebral palsy) or
Treatment Principles
e basic principles of observing or bracing smaller, exible curves and surgically fusing larger, more rigid curves
469
470 PEDIATRICS
AB
C D
FIG. 28.1 Progression of scoliosis after skeletal maturity in a patient with cerebral palsy. (A) At age 15, curve
measures 75 degrees. (B) At age 18, curve measures 115 degrees. (C) At age 23, curve measures 143 degrees.
(D) The patient is unable to be comfortably positioned in her wheelchair.
in adolescent idiopathic scoliosis apply to the treatment of
neuromuscular scoliosis, although with less aggressive parameters. Observation alone is employed until curves begin to
cause functional impairment. Bracing can be a temporizing
measure, used primarily to provide sitting support while
the patient grows. Eventually, many of these patients require
surgical stabilization with a spinal instrumentation and fusion
procedure.
Nonoperative Treatment
Medical Treatment
Spinal Muscular Atrophy
Before the 1990s, there was no clear molecular target for SMA
diseases. As a result, various medications were tried in an
Chapter 28 Neuromuscular Scoliosis 471
FIG. 28.2 Severe spinal deformity can lead to skin maceration on concave
side of curvature and pressure sores on convex side.
o-label fashion that found dierent degrees of success in
other diseases causing muscle weakness, such as amyotrophic
lateral sclerosis. Randomized placebo-controlled trials have
been conducted to investigate the ecacy of several medical
treatments for SMA, including creatine, phenylbutyrate, gabapentin, and thyrotropin-releasing hormone.
2–5
None of these
compounds has proven to be an ecacious drug treatment for
SMA.2 e discovery of the survival of motor neuron (SMN)
gene has resulted in the development of animal models to test
new therapies. e main objective of these therapies is to
increase the expression of the SMN protein. ese RNA-based
molecule therapies along with gene therapy have shown
promise in animal models and are currently in the preclinical
testing phase.6 However, as of yet these therapies have not
been eective in human trials.
Cerebral Palsy
Several medical therapies have been investigated for the treatment of spasticity in patients with cerebral palsy (CP). Botulinum toxin has gained a growing acceptance as a treatment
of upper and lower limb spasticity. Initial reviews of the literature by the Cochrane Collaboration and others yielded
inconclusive evidence that could neither conrm nor deny the
ecacy of botulinum toxin in the treatment of spasticity.7
Inclusion of more recent randomized controlled trials (RCTs)
into the analysis has provided evidence that supports the use
of botulinum toxin to provide a time-limited benet to
decrease muscle tone in children with upper and lower limb
spasticity associated with CP.8 In a study of 16 children with
CP, botulinum toxin A injections into the gastrocnemius
muscle decreased spasticity and improved ankle range of
motion (ROM), pattern of walking, and reduced energy consumption, providing improvement in function. e evidence
trend is in favor of using this therapy to reduce spasticity early
in the management of CP, although there is little evidence that
this has a role in preventing or treating scoliosis.
Intrathecal baclofen is a well-established treatment that
has been shown to provide signicant benets in controlling
spasticity in patients with CP. Intrathecal baclofen has been
shown to reduce the need for orthopaedic lower extremity procedures and the rate of postoperative complications
associated with these procedures.9 Concerns have been raised,
however, regarding its impact on the progression of scoliosis
in patients with spastic quadriplegia. In a retrospective review,
Ginsburg and Lauder10 found a six-fold increase in the rate
of scoliosis curve progression at 2-year follow-up in a group
of 19 quadriplegic CP patients with spasticity. Caird and colleagues11 showed a signicantly higher rate of complications
associated with posterior spinal fusion and instrumentation
in a group of 20 CP patients with spasticity with intrathecal
baclofen pumps compared with a matched control group. is
study was limited by its relatively small sample size and lack of
a control group. Shilt and colleagues12 found no dierence in
curve progression at 3-year follow-up between 50 CP patients
treated with intrathecal baclofen and 50 matched control CP
patients. In a review of the literature, Scannell and Yaszay
suggested that no conclusive evidence exists to support the
hypothesis that intrathecal baclofen pumps lead to worsening
of the scoliosis in CP patients.13 erefore, based on the current
evidence, no signicant conclusions can be drawn about the
impact of intrathecal baclofen pumps on the progression or
treatment of spinal deformity in patients with CP. Baclofen
can provide signicant relief of spasticity, and this evidence
must be considered in the context of any potential side eects.
Duchenne Muscular Dystrophy
Advances in general care, glucocorticoid treatment, noninvasive ventilatory support, cardiomyopathy management, and
scoliosis management have signicantly changed the course
of Duchenne muscular dystrophy (DMD). Survival into
adulthood is now a realistic expectation for many patients
who receive optimal treatment.13 Although gene-based and
cellular-based therapies are currently under development for
the treatment of DMD, the ecacy of glucocorticoid steroids
has been evaluated by several randomized controlled trials.
In their Cochrane review and meta-analysis, Manzur and
colleagues
function and strength are improved in the short term (6
months to 2 years) with corticosteroid therapy. e authors
based their conclusion on six RCTs and observed that the
most eective prednisolone dose seemed to be 0.75 mg/kg
per day, given daily.14 Markham and colleagues16 showed that
glucocorticoid therapy provides the added benet of retard-
ing the anticipated development of ventricular dysfunction
if begun before ventricular dysfunction in their series of 14
DMD patients treated with steroids compared with 23 DMD
14,15
concluded that there is evidence that muscle
SECTION
IV
472 PEDIATRICS
AB
FIG. 28.3 Bracing in neuromuscular scoliosis is often poorly tolerated. Although it provides modest correction,
as shown in these radiographs, rigid bracing may lead to excessive skin pressure in patients who cannot
actively pull away from the brace.
patients treated without steroids. Shapiro and colleagues17
evaluated 85 DMD patients who were not receiving steroids,
nding that 97% had progression of their scoliosis of more
than 10 degrees aer becoming wheelchair dependent, regardless of age. Based on their ndings, Shapiro et al.17 advocated
for spinal fusion in the majority of patients aer the curve had
progressed beyond 20 degrees and who were not on steroid
therapy to avoid future cardiac and pulmonary complications.
Conversely, Lebel et al.18 found a reduced incidence of scoliosis development and need for spinal fusion in patients treated
with long-term glucocorticoids.
Genetic and Family Counseling
Because of the complexity of the medical and psychosocial issues
associated with neuromuscular disorders and spinal deformity,
care needs to be coordinated with a multidisciplinary team. e
primary care physician should be well informed of all orthopaedic issues and play a central role in managing care. Psychosocial
support for patients and parents is also vital. Patient advocacy
groups have proven to be very useful in helping families cope
with the illness and associated surgical care. Physicians may
wish to provide information regarding clinical trials or refer
families to clinical trial websites (www.clinicaltrials.gov pro-
vides a current listing of open clinical trials). Patients and
parents may need to be referred for genetic counseling to
conrm the patient’s diagnosis and aid in family planning.
Bracing
Bracing is a controversial treatment method in idiopathic and
neuromuscular scoliosis. Bracing in neuromuscular scoliosis
may be used for postural support, although there is limited
evidence of its ecacy in limiting curve progression (Fig.
28.3). e etiology of the patient’s scoliosis and the patient’s
muscle tone have an impact on the practicality of brace treatment. Patients with spastic disorders generally do not tolerate
rigid brace treatment, whereas patients with accid paresis are
more apt to be compliant with brace treatment. e type of
orthoses may play a role in the outcome of the treatment.
Kotwicki and colleagues19 followed 45 nonambulatory
patients with neuromuscular scoliosis treated with a suspension trunk orthosis (STO) and found that the STO slowed
curve progression in 23 patients. e STO construction functions contrary to the classic thoracolumbosacral orthosis
(TLSO), with the STO not resting against the patient’s pelvis
but rather directly against the seat. e evidence supporting
STO use to prevent curve progression is limited, however, and
skin intolerance found in 36 patients complicates its clinical
practicality. Although there is limited research on the results
of the STO brace, there are numerous studies investigating the
TLSO brace. In a study of 15 patients, Shoham and colleagues20
found that a TLSO reduced scoliotic deformity and pelvic
obliquity leading to reduced sitting pressure. ese results are
contrary to other studies reported in the literature. In a study
of 23 patients, Miller and colleagues21 followed 23 patients
with CP who wore a rigid Wilmington TLSO for an average
of 67 months and concluded that the bracing did not slow
progression of their deformity. Olafsson and colleagues22 followed 90 patients with various neuromuscular conditions
treated with a so Boston orthosis for an average of 3 years
aer brace treatment. ey concluded that brace wear was
indicated only in a limited subset of patients—ambulatory
patients with hypotonia and short thoracolumbar curves (<40