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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6025_Библиотеки_им_академика_М_И_Перельмана
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21 Pediatric Spinal Deformity
21.1 General Considerations
I. Classi cat ion (Table 21.1).
21.2 Idiopathic Adolescent Scoliosis
I. Et io lo g y.
A. Ne u r o m u s cu la r.
1. Changes in the muscle ber types and muscle spindles have been
demonstrated.
2. Increased calmodulin level, which is responsible for the regulation of muscle
cont raction, and decreased m elatonin levels (calm odulin antagonist) have
been demonstrated in patients with idiopathic adolescent scoliosis (IAS).
B. Ho r m o n a l.
C. Co n n ec t ive t is su e.
1. Elastic and collagen bers are the primary elements that support the spine.
2. Abnormalities of the collagen/proteoglycan in the intervertebral disks have
also been reported.
D. Gen etic: 5:1 fem ale p repon derance (> 10° cu rves), fam ilial (20 tim es greater
likelihood in fam ilies) w ith monozygotic tw in concordance rate of 73%, genet ic
(sex-linked trait with incomplete penetrance and variable expressivity).
E. Mela t o n in o r s e r ot o n in a b n or m a lit ie s .
II. Anatom ical ch aracte r ist ics .
A. La t e ra l cu r va t u re o r d efor m it y in t h e co r on a l p la n e .
B. De cr e a s ed t h o r ac ic k yp h os i s o r t h o r ac i c h yp ok yp h o s is ( d e fo r m it y in t h e s a git t al
plane):
1. Earlier accelerated spinal growth as compared with normal individuals may
be related.
C. Ve r t eb ra l r ot a t io n : t h e s p in ou s p ro c e ss r ot at e s t ow a rd t h e co n ca v it y (d efo r m it y
in the axial plane) an d cau ses rib hum p.
D. Pat tern s of th oracic scoliosis:
1. King classi cation (Table 21.2): helps determine fusion levels for surgery
(not all curves t into this classi cation).
2. Lenke classi cation: newer, more extensive, classi es the curves based on
curve type, coronal lumbar modi er, and thoracic sagittal pro le (Fig. 21.1).
a. Four series of plain lm spine radiographs are used: upright
posteroanterior, lateral, supine right-bending, and supine left-bending.
b. Curves can be classi ed into six types:
(1) Type 1: Main thoracic (MT).
(2) Type 2: Double thoracic (DT).
(3) Type 3: Double m ajor (DM).
(4) Type 4: Triple major (TM).
227
228
21 Pediat ric Spinal Deform ity
Ta b l e 2 1 . 1 Classi c a t io n o f p e d iat ric sp in a l d efo rm it y
Cl a s s i f c a t i o n Ty p e S u b t y p e s
Non st ruct ural Post ural
Scia t ic
In a m m a t o ry
Co m p e nsa t o r y
St ru ct ura l Id io pa t hic In fa nt ile (< 3 ye ars )
Ju v e n i le ( 3 – 1 0 y e a r s )
Ad o l e s c e n t ( 1 0 ye a r s t h r o u g h
maturity)
Ne u rom uscu la r
Ne u rop a t hic
Myo p a t h ic
Ce re bra l p als y
Syrin go m ye lia
Polio m yelit is
Spin al m uscula r a t ro phy
Fr ie d r e ic h ’s a t a x ia
Ar t h r o g r y p o s is
Mu s c u la r d ys t ro ph y
Myo t on ia d yst ro p hica
Co ng e nit a l Dia s te m at o m ye lia , sp in a
bi da, hemivertebra, wedge
vert ebra, u n se g m e nt ed
bar with contralateral
hemivertebra, block vertebra
Ne u ro b rom at osis
Me s e n ch ym a l d iso rd e rs Ma r fa n’s s yn dro m e
Eh l e r s – D a n l o s s y n d r o m e
Rh e u m a t o id d is e a se
Tr a u m a
Ext r a s p i n a l c o n t r a c t u r e Bu r n s
Th ora cic su rg e ry
Osteochondral dystrophies
In fe c t io n
Me t a b olic d iso rd ers
Re l a t e d t o lu m b o s a c r a l j o i n t
Tu m o r s
21 Pediat ric Spinal Deform ity 229
Ta b l e 2 1 . 2 Ki n g c l a s s i c a t i o n s y s t e m
Ty p e King classifcationNo tes
Doub le m ajor right t horacic
and left lumbar
Rig h t t h o r a cic a n d
compensatory left lum bar
ILumbar curve is larger than
the thoracic curve
II Th ora cic c u r ve is la rg e r
than the lumbar curve
Rig h t t h o r a cic III Le f t lu m b a r c u r ve d o e s n o t
cross the midline
Rig h t t h o r a co lu m b a r IV
Doub le t horacic V
Fi g . 2 1 . 1 Le n k e c l a s s i c a t i o n . ( F r o m Le n k e LG , B e t z R R , H a r m s J , e t a l . A d o l e s c e n t i d i o p a t h i c s c o l i o s i s :
a new classi cation to determine extent of spinal arthrodesis. J Bone Joint Surg Am 2001;83-A:1169–
1181. Reproduced with permission.)
230
21 Pediat ric Spinal Deform ity
(5) Type 5: Thoracolum bar/lum bar (TL/L).
(6) Type 6: Thoracolum bar/lum bar–m ain thoracic (TL/L-MT).
c. The major curve is the largest curve.
d. Minor curves are then evaluated for structural criteria.
(1) Str u ctu ral cu r ve: coron al p lan e r igidit y > 25 ° u p on sid e-ben din g or
kyphosis > 20° on sagitt al radiograp h s.
e. Spinal arthrodesis should include only the major curve and the structural
minor curves.
f. A lu m b a r coron al m od i er is d et erm in e d.
(1) A central sacral vertical line (CSVL) is drawn vertically from the
midpoint of S1.
(a) Modi er A: the CSVL traverses between the pedicles of the apical
vertebrae.
(b) Modi er B: th e CSVL lies between th e medial border of th e
concave pedicle and the lateral edge of the apical vertebral body.
(c) Modi er C: the CSVL is not adjacent to the lateral border of the
apical vertebral body.
g. The last component involves assessment of the sagittal thoracic alignment.
(1) The Cobb angle from T5 to T12 is m easured.
(2) If the Cob b angle is +10 to +40°, a norm al m odi er is assign ed .
(3) A m in u s sign in d icates a Cobb angle < 10° (hyp okyph otic cu r ve).
(4) A p lus sign indicates a Cobb angle > 40° (hyperkyp h otic).
h. Forty-two di erent types of curves are possible because type 5 and 6
curves are associated with a lum bar coronal m odi er of “C.”
3. Isolated lumbar or thoracolumbar curves.
III. Nat u ral h ist o r y a n d p ro gn os is .
A. Prevalence: 25 /1,000 (2.5%) exh ibit > 10° cur ves an d (0.4%) 4/1,000 exh ibit
> 20° cur ves (Table 21.3).
IV. Diagn osis.
A. Scr e e n in g: ge n e r a lly o ccu r s in sch oo l ch ild re n age d 1 0 t o 1 4 :
1. Leads to a substantial number of referrals.
2. One-third of all referrals have scoliosis to some degree.
3. Genetic testing is available to determine the risk of curve progression in
patients with AIS:
a. ScoliScore (Transgenomic, Inc.).
(1 ) Though t t o determ ine th e r isk of cu r ve progression past 40°.
(2) Should be used as an adjunct to clinical exam ination and
radiographic ndings.
(3) There is mixed evidence regarding the e cacy of this genetic test.
B. His t o r y: a ge , ge n d e r, o n se t o f m e n ar ch e , p a i n , fa m ily h is t o r y.
1. Pain occurs in up to 30% of patients with AIS.
2. Peak growth occurs in girls at age 11 to 12 and in boys at age 13 to 14.
C. Ph ys ic a l e x a m in at io n :
1. Observation.
a. Asymmetry of shoulder level, breasts, waist, or pelvis.
b. Protruding scapula or ribs.
c. Loss of thoracic lordosis.
Ta b l e 2 1 . 3 Fa c t o r s c o n t r i b u t i n g t o c u r v e p r o g r e s s i o n
21 Pediat ric Spinal Deform ity 231
Cu r ve
magnitude
Th e g re at e r t h e a n g u la t io n a n d ro t at io n , t h e g re at e r t h e t e n d e n c y
for p rogre ssion. Fo r e xam ple, a 2 0 ° curve has a 20% like liho o d o f
progression, and a 40° curve has a 60% likelihood of progression.
Ag e Yo u n g e r a g e is a m o r e im p o r t a n t p r o g n o s t i c f a c t o r t h a n g e n d e r
or family history. Ninety percent of spinal growth has occurred at
puberty; however, this age has the highest risk of progression.
Ris se r s co r e A sco r e o f 1 o r le s s h a s a h ig h e r like lih o o d o f p r o g re s sio n .
Cu r ve size Sho r te r cu rve s p ro g re s s m o re
Lo c a t i o n Th e l o w e r t h e c u r v e i s i n t h e s p i n a l c o l u m n , t h e g r e a t e r t h e
like lih o od o f p rog ression (t hora cic < lum b a r).
Fle xib ilit y St i e r cu rve s in im m a t u re in d ivid ua ls a n d m o r e e xib le cu rve s in
mature individual are more likely to progress.
Ge n de r Girls a re m o re co m m only a e cte d, e spe cially fo r larg e r cu r ve s.
Fa m i l y h i s t o r y
Sle n d er sp in e
d. Adams forward bend test.
(1 ) The p atien t ben ds at t h e w aist to 9 0°.
(2) During the process, assess for asym metry in bending and rotational
deformity of thoracic and lumbar curves.
2. Measurements.
a. A scoliometer is used to measure the rib hump (rotational deformity on
for w a r d b en d in g).
b. A plumb line dropped from C7 indicates coronal balance relative to the
gluteal cleft.
c. Leg length discrepancy.
3. Neurological examination.
a. Deep tendon re exes.
b. Abdominal re exes.
(1) Check for symm etrical um bilical movem ent upon lateral to medial
light stroke on the abdom en .
(2) Asym m etrical m ovem ent correlates with neural axis pathology.
D. X-ray exam in at ion :
1. The Cobb angle (Fig. 21.2) is used to d ete r m ine the m agn it u d e of t h e cu r ve.
Th e u p p er a n d low e r e n d ve r teb ra e o f e a ch cu r ve a re id e n t i e d . A lin e is
drawn at the upper end of the cranial end vertebra along the end plate or
by marking the upper or lower m argin of the pedicles. A line is then drawn
232
21 Pediat ric Spinal Deform ity
ab
Fi g . 2 1 . 2 ( a , b ) Co bb a n gle a na lysis fo r m a n ual a d o le sce n t id io p at h ic sco lio sis m e as u r e m en t s.
at the lower end of the caudal vertebra of the curve, using the end plate or
pedicles. A right angle to this line is then drawn. The angle to be measured is
the angle formed by the two lines at the end vertebrae.
2. Bone age determination can be useful to help determine the risk of spinal
curve progression.
a. Risser sign (Fig. 21.3): ossi cation of the iliac epiphysis progresses from
the anterior iliac spine posteriorly. A Risser 0 has the least ossi cation
and greatest risk of progression, whereas a Risser 5 indicates the
epiphysis has fused with the iliac crest, and the risk of progression is
minimal.
b. A ring apophysis fusion indicates cessation of all vertebral body growth
potential.
c. Left wrist and hand: the X-ray is compared with standards in the
Greu lich a n d Pyle atla s.
E. Pulm on ar y fun ct ion test : > 70° cu r ves have d e creased vital capacit y,
particularly with hypokyphosis.
F. In d i c a t i o n s fo r m a g n e t i c r e s o n a n c e i m a g i n g ( M R I) :
1. Neurological abnormalities.
21 Pediat ric Spinal Deform ity 233
a
Fig . 2 1 . 3 ( a – d ) Th e Risse r Sig n: Os s i ca t io n
of the iliac apophysis progresses from the
anterior iliac spine posteriorly. A Risser 0 has
the least ossi cation and greatest risk of progression, while a Risser 5 indicates the apophysis has fused with t he iliac crest and the risk of
progression is minimal.
b
c
d
234
21 Pediat ric Spinal Deform ity
2. Congenital vertebral abnormalities.
3. Juvenile and infantile onset.
4. Rapid progression.
5. Cutaneous manifestations of dysraphism.
V. M a n a g e m e n t .
A. Go a ls o f t r e at m e n t .
1. Prevent progression and maintain balance.
2. Maintain respiratory function.
3. Reduce pain and preserve neurological status.
4. Cosmesis.
B. No n op e r at ive t r e at m e n t .
1. For most patients with scoliosis, progression may not be severe enough to
warrant treatment.
2. Obser vat ion is ind icated for curves < 25° in im m at ure p atie n ts and > 50° in
mature patients.
a. Obtain X-ray 3 months after the rst visit and then every 6 to 9 months
for cu rves < 20 ° an d ever y 4 to 6 m on t h s for cur ves > 20°.
b. A sign i can t ch a n ge is d e n e d as p rogression of > 10° in cu rves < 20° an d
> 5° in cu r ves > 20°.
3. Exercise is indicated only as an adjunct treatment, especially for patients
with obesity, back pain, lumbar hyperlordosis, exible kyphosis, and trunk
and extremity muscle tightness.
4. Or t hosis: cur ve > 30 to 45° ( rst visit) an d > 25° w it h docu m en te d
progression in immature patients (Risser 3 or less):
a. Not for cervicothoracic curves and hypokyphotic thoracic curves.
b. The goal is to prevent progression: ~ 85% of compliant patients
demonstrate progression cessation and improve (~ 50% correction), but
m ost patien ts ten d to retu r n w ith in 5° of the or igin al cu rve after t he
brace treatment is ceased.
c. Protocol: the patient must wear the brace 23 hours a day until 2 years
after menarche or Risser 4 and be weaned o in 1 year (part-time wear is
also reported).
d. Orthosis types:
(1) Thoracolum bar sacral orthosis (Boston overlap).
(a) Appropriate up to T8 apex.
(b) All curve types.
(c) Medium compliance.
(2) Bending brace (Charleston).
(a) Thoracolum bar an d lum bar cu rves (25–35°).
(b) Best com pliance.
(3) Mehta cast.
(a) Treatment option for very young children.
(4) Cervico Thoracolum bar Sacral Orthosis (CTLSO) (GB Orthopaedics).
(a) Thoracic curves with apex above T7.
(b) Low compliance.
5. Electrical stimulation treatment has been abandoned.
C. Op e r at ive t r e at m e n t .
1. Indications for surgery.
a. Progressive cu r ves > 40 to 45° in grow ing ch ild ren (Fig. 21.4).
b. Failure of bracing.
c. Progressive cur ves beyond 50° in ad u lts.
21 Pediat ric Spinal Deform ity 235
Fi g . 2 1 . 4 A 1 7 - y e a r - o l d g i r l w i t h
adolescent idiopathic scoliosis.
(a) Anteroposterior radiograph
demonstrating a 49° right thoracolumbar curve with maintenance of the overall coronal
balance. (b) Lateral radiograph
demonstrates relatively preserved
sagit tal alignment. Postoperative
(c) anteroposterior and (d) lateral
radiographs with an anteroposterior release and fusion.
ab
c d
236
21 Pediat ric Spinal Deform ity
2. Goals of surgery.
a. Spinal and pelvic balance is more important than curve correction.
b. Prevent respiratory compromise.
c. Prevent back pain.
d. Cosmesis.
3. Operative management based on the Lenke classi cation.
a. Type 1: posterior fusion and instrumentation (PFI) is favored.
(1) Lower extent vertebrae (LEV) is controversial.
b. Type 2: require PFI.
(1) The proximal fusion level (T2 or T3) is determ ined by the size of the
proximal thoracic curve and shoulder alignment.
(2) LEV is controversial.
c. Type 3: PFI is warranted.
d. Type 4: Rare and warrants fusion of the proximal thoracic curve, main
thoracic curve, and thoracolumbar/lumbar curves.
(1) May require an anterior release for rigid curves.
e. Type 5: Only require fusion of the thoracolumbar/lumbar curve given
that it is structural (anterior or posterior).
f. Typ e 6: PFI of t h e m ajor t h o r a colu m bar / lu m b a r an d t h e m in or t h oracic
curve.
VI. In st r u m en t at io n .
A. Co n te m p or ar y syst e m s .
1. Vertical Expandable Prosthetic Titanium Rib (VEPTR) (Synthes Spine) (Fig. 21.5).
a. Received humanitarian device exemption status to treat chest wall and
spinal deform ity associated with thoracic insu ciency syndrom e.
b. Maximizes lung volume by expanding the rib cage and enabling grow th
and correction of the spinal deformity.
c. Long-term outcom es with VEPTR are pending, because sagittal deform ity
remains unaddressed.
2. Growing rod instrumentation.
a. Used in adolescent children to enable normal growth of the spine while
addressing spinal deform ity correction.
b. Requires lengthening at periodic intervals.
VII. Su rgical t e ch n iq u e .
A. In t r ao p er a t ive cell save r s ys t e m .
B. In t r a o p e r at iv e n e u ro m o n it or in g a n d w a ke - u p t e s t o r m o t o r e vo ke d p ot e n t ia ls .
C. Fu s i o n t e ch n iq u e:
1. Subperiosteal dissection out to the tips of the transverse processes.
2. Decortication, facet cartilage excision.
3. Iliac crest autograft or rib graft from thoracoplasty.
D. Inst ru m e n tat ion te ch niqu es: m ost d efor m it y corre ct ions are n ow per form ed
using the pedicle screw–based systems.
VIII. Post su r g ic a l ca re , ou t com es, a n d com plic a t ion s.
A. Br acin g a ft e r s u rge r y is n ot r e q u ir e d .
B. Pa t ie n t s a r e s lo w ly a d va n ce d in t h e ir a ct iv it ie s u n t il fu ll r e co ve r y i n 6 t o 1 2
months.
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