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21 Pediat ric Spinal Deform ity 247
ab
c
d
Fi g . 2 1 . 8 Im a g e s o f a 4 4-ye a r-o ld m a n w it h n e u ro b ro m a t o sis wh o p re se n t ed wit h se ve re k yp h o s c o -
liosis an d pa rap le g ia. A sim ult a n e ous ant e rio r and p o st e rior app ro ach wa s p e rfo rm ed on t h is pa t ien t t o
combine anterior and posterior procedures. The steps of the procedures included a posterior osteotomy, anterior vertebrectomy, spinal cord decompression, anterior fusion with strut grafting, posterior
compression instrumentation, and anterior instrum entation, in this order. (a) Preoperative magnetic
resonance im age showing 170° kyphosis with cord compression. (b) Computed tomographic (CT)
myelogram demonstrating severe kyphotic deformity with signal cuto distal to the T9 vertebra.
(c) Int ra o p e rat ive p hotogra ph sho wing an t e rior vert e b re ctom y, bu lar strut graft , a nd an t e rio r inst rumentation. (d) Postop erative radiograph showing 65° of kyph osis.
(b) Com bined anterior and posterior fusion with instrum entation in
severe xed deform ities.
5. Myelomeningocele.
a. Congenital.
(1) It is generally not recom m ended to correct deform ity at birth
because of the lack of bone stock and associated problems.
b. At 3 to 5 years of age, posterior resection followed by correction of kyphosis.
(1) Posterior instrumentation should be applied two to three vertebrae
proximal to the apex.
(2) Followed by 6 to 9 months of bracing.
248
21 Pediat ric Spinal Deform ity
ab
cd
Fi g . 2 1 . 9 Pre o p e rat ive rad iogra p hs of an 18-year-o ld wo m an wit h Sch e ue rm a nn’s kyp h o sis. (a)
An t e r o p o s t e r io r r a d io g r a p h d e m o n s t r a t i n g n o co r o n a l p la n e d e f o r m i t ie s . (b) Lat eral rad iograph m easuring 80° of kyphosis from T1 to T12. (c,d) Be ca u se of t h e p atient ’s re lative e xibilit y, a n all post e rior
approach was performed, restoring her sagittal balance.
21 Pediat ric Spinal Deform ity 249
6. Developmental or paralytic kyphosis.
a. The deformity is progressive because the extensors of the spine
(sacrospinalis and quadratus lum borum muscles) rotate anteriorly to
increase exion force.
b. Treatment:
(1) Bracing is indicated for young patients with mild deform ity.
(2) Posterior fusion involving long fusion to the sacrum .
(3) Anterior release and fusion followed by posterior fusion w ith
compression instru m entat ions.
7. Posttraumatic kyphosis.
a. Acute or late secondary to severe compression fractures, burst fractures,
or fracture-dislocations.
b. More common for unstable fractures after conservative treatment.
c. Symptom s include deform ity, pain, and neurological de cit.
d. Treatment:
(1) Observation and conservative treatm ent if the deform ity causes mild
pain that is controllable.
(2) Surgery is most com monly done by a com bined anterior and
posterior fusion.
(a) Anterior decompression with anterior instrum entation alone
may be done if adequate correction has been achieved and the
instrum entation is stable.
(b) Posterior-only tran spedicu lar osteotom y m ay be done if t h ere is
no neurological compression.
8. Postsurgical kyphosis.
a. Usually observed after laminectomy for spinal cord tumors and
syringomyelia.
b. Fusion is recommended at the time of surgery when extensive
lam in ectom y has been perform ed.
c. Severe deformity is approached with a combined anterior and posterior
fusion .
9. Infectious kyphosis (Fig. 21.10).
a. Infection may be caused by tuberculosis and pyogenic osteomyelitis.
b. The thoracolumbar junction is most commonly a ected.
c. Treatment:
(1) Antibiotic treatm ent includes long-term intravenous antibiotics and
bracing.
(2) Anterior debridem ent and fusion indications:
(a) Unresponsive to m edical treatment.
(b) Multiple-level involvem en t.
(c) Spinal cord compression.
(d) Presence of an abscess.
(3) Progressive kyphosis needs anterior and posterior procedures.
10. In ammatory kyphosis (ankylosing spondylitis).
a. Characterized by loss of a lumbar lordosis and increased cervical and
thoracic kyphosis.
b. Prim ary location of disabling deformity should be assessed.
250
21 Pediat ric Spinal Deform ity
ab
Fi g . 2 1 . 1 0 A 6 5 - ye a r - o ld m a n w it h o ld t u b e r c u lo s is w h o h a d a p o s t e r io r f u s io n 4 0 ye a r s p r io r .
Despite se ve re kyphosis, t h is p at ie n t was a m bula t o r y wit h m in im al sym p t o m s. (a) Lateral radiograph
showing severe angular kyphosis. (b) Sag it tal T2-we ighted m ag net ic reson ance im ag e (MRI) sho wing
an old granuloma and spinal cord compression at the apex of the kyphotic deformity.
c. Hip exion contracture should be corrected rst.
(1) May avoid the need for spinal surgery.
d. Lumbar osteotomy is indicated for a signi cant loss of lumbar lordosis.
(1) Types of osteotom y.
(a) Smith–Petersen/Ponte opening wedge extension osteotomy.
i. Typically perform ed at L2–L3 an d L3–L4 junction followed by
inst rum entat ion an d fusion .
ii. Th e angle of correction corresponds to the spine exion
deformity on standing.
iii. The apex of th e osteotom y sh ould be an terior to the neural
tube and at the junction of the posterior longitudinal ligament
and intervertebral disk.
(b) Pedicle subtraction osteotomy.
i. Does not length en the anterior colum n.
ii. A closing wedge osteotomy shortens the vertebral colum n and
is safer neurologically.
iii. Posterior resection of the lam ina, pars, and pedicles is
performed followed by a vertebral wedge resection.
11. Flat back syndrome.
a. Etiologies.
(1) Distraction of the lum bar spine (Harrington instrum entation).
(2) Lum bar fusion with loss of lordosis.
b. Clinical ndings.
(1) Sagittally im balanced posture.
(2) Back pain with referred pain down to legs.
(3) Transition syndrom e above and below kyphosis.
(4) Compensatory hip and knee exion contractures.
c. Treatm ent.
(1) Conservative treatm ent with nonsteroidal anti-in am matory drugs,
21 Pediat ric Spinal Deform ity 251
physical therapy, range of motion exercises, pain management.
(2) Surgery.
(a) Lum bar osteotom y to balance the sagittal contour of the spine.
i. Pedicle subt ract ion osteotomy.
ii. Sm ith–Petersen osteotom y.
Sugg este d Reading
Ku k l o TR, P o t t e r BK, S ch r o e d e r TM , O ’Br i e n M F. Co m p a r i s o n o f m a n u a l a n d d i g it a l m e a -
sure m e n t s in adolescen t idiopat h ic scoliosis. Spin e 200 6;3 1(11 ):12 40–1246
Le n k e LG. Le n k e c la s s i c a t i o n s y s t e m o f a d o l e s c e n t id i o p a t h i c s c o li o s is : t r e a t m e n t r e c o m -
m en dations. Inst r Course Lect 2005;54:53 7–542
Le n k e LG, Be t z RR, Ha r m s J, e t a l . Ad o l e s ce n t id i o p a t h i c s c o li o s i s: a n e w c la s s i ca -
tion to determine extent of spinal arthrodesis. J Bone Joint Surg Am 2001;83-A(8):
1169–1181
Le n k e LG, Ed w a r d s CC II, Br i d w e l l KH . Th e Le n k e c la s s i c a t i o n o f a d o l e s ce n t i d i o p a t h i c
scoliosis: how it organizes curve patterns as a template to perform selective fusions of
the spin e . Spine 2003;28(20):S19 9–S207
Lu n d i n e KM , Le w i s SJ, Al - Au b a i d i Z , Al m a n B, H o w a r d AW . P a t i e n t o u t c o m e s i n t h e o p -
erative and nonoperative management of high-grade spondylolisthesis in children. J
Ped iatr Orthop 2 014;34(5):4 83–489
O’Neill KR, Len ke LG, Br idw ell KH, et al. Clin ical an d ra d iograph ic outcom e s after 3-
colu m n osteotom ies w it h 5-year follow - u p . Spin e 2014;3 9(5):424 –432
22 Pediatric Cervical Spine Disorders
22.1 General Considerations
I. Develop m en t a l an at om y.
A. At las .
1. Neurocentral synchondrosis forms at 6 to 24 months.
a. Fusion begins at 4 to 6 years.
b. Posterior synchondrosis fuses at 5 years.
B. De n s.
1. Two primary ossi cation centers coalesce at 1 to 3 months of age.
2. Separated from the vertebral body by a dentocentral synchondrosis that
fuses at 6 to 8 years.
C. No r m a l va r ia n t s.
1. Posterior bi d C1 arch.
2. Bipartite superior articular surface of the atlas.
3. Pseudonotch of the atlas.
4. Absence or partial absence of the posterior arch of the atlas.
5. Posteriorly displaced spinolaminar line of the axis.
6. Posteriorly angulated dens.
7. Pseudosubluxation of the axis (< 10 years old).
II. Ph ysical exa m in at ion .
A. Lim it e d r a n ge o f m ot io n .
B. To r t ic o llis .
C. Fa c ia l a sy m m e t r y.
D. Associated abnor m alit ies.
1. Scoliosis, renal, cardiac, or other head and neck anomalies.
III. Diagn o st ic e va lu at io n (Fig. 22.1 and Fig. 22.2).
A. A e xion -e xt e n sio n view is ve r y im p o r t a n t t o a s s e s s st ab ilit y.
B. Cr it ic a l m e a su r e m e n t s o n e x e d l a t e r a l v ie w :
1. Atlantoaxial interval: 4.5 mm (children), compared with 3 mm (adults).
a. From the posterior aspect of the anterior C1 arch to the odontoid process.
2. Space available for spinal cord (SAC): 13 mm.
a. Determines the minimal amount of space required within the spinal
canal to accom m odate the spinal cord.
252
IV. Speci c d isorders.
A. Bas ila r in vagin at io n (Fig. 22.3).
1. Deformity of the bones at the base of the skull at the margin of the foramen
magnum.
a. The odontoid is migrated cephalad.
22 Pediat ric Cervical Spine Disorders 253
Fi g . 2 2 . 1 La t e r a l i l l u s t r a t i o n o f t h e n o r m a l r e l a t i o n s h i p o f t h e C 1 – C 2 a r t i c u l a t i o n d e m o n s t r a t i n g t h e
atlantodens interval (ADI) and the space available for cord (SAC).
2. Types.
a. Primary.
(1) Congenital.
(2) Associated with other ndings.
(a) Atlanto-occipital fusion.
(b) Hyp oplasia of atlas.
(c) Bi d posterior arch of the atlas.
(d) Odontoid abnormalities.
(e) Klippel–Feil syndrom e.
b. Secondary.
(1) Developm ental condition with softening of the base of the skull.
(2) Associated with other clinical disorders.
(a) Osteom alacia.
(b) Rickets.
(c) Paget’s disease.
(d) Osteogenesis imperfecta.
(e) Renal osteodystrophy.
(f) Rh e u m at oid a r th rit is.
(g) Neuro brom atosis.
(h) Ankylosing spondylit is.
(i) Ach on droplasia.
254
22 Pediat ric Cervical Spine Disorders
a
b
Fi g . 2 2 . 2 ( a ) In exion, the sp ace ava ilab le for cord (SAC) m ay de cre ase be t ween t he p o st e rior asp e ct of
the dens and the anterior aspect of the C1 posterior ring. (b) In e xte n sio n , t he SAC m ay d ecre a se bet we e n
the posterior aspect of the dens and the anterior aspect of the C2 lamina or the foramen magnum.
3. Clinical ndings.
a. Commonly become symptomatic in second and third decades.
(1) Short neck.
(2) Asym m etric face.
(3) Torticollis.
(4) Weakness/paresthesias.
(5) Cranial nerve palsies.
(6) Cerebellar signs (unsteady gait and nystagm us).
(7) Pain (head and neck).
22 Pediat ric Cervical Spine Disorders 255
ab
c
Fi g . 2 2 . 3 An 1 8 - ye a r -o ld m a n wit h o s t e o g e n e s is im p e r fe c t a a n d b a s ila r i nva g in a t io n p re s e n t e d wit h
unrelenting headache, neck pain, and myelopathy. (a) La t era l rad io g ra p h de m o nst rat in g b asila r in vag i-
nation with the odontoid protruding into the foramen magnum. (b) Ma g ne t ic re so n a nce im a g ing sca n
showing the odontoid indenting the brain stem. (c) Postoperative lateral radiograph showing a triple
wire technique and posterior occipital–cervical fusion.
(8) Syncope and dizziness (vertebral artery compression).
(9) Seizures/hydrocephalus (cerebrospinal obstruction).
4. Treatment.
a. Posterior impingement.
(1) Suboccipital craniectomy and decompression of the posterior ring of
C1 w it h p o st e r i o r s t a b i l i z at io n .
b. Anterior impingement.
(1) Mobile odontoid:
(a) Occipitocervical fusion in extension is recom m ended.
256
22 Pediat ric Cervical Spine Disorders
(2) If the odontoid cannot be reduced, anterior excision of the odontoid
and posterior stabilization are recommended.
B. Kl ip p el– Fe i l s y n d r o m e (Fig. 22.4).
1. Congenital fusion of cervical vertebrae.
2. Failure of normal segmentation of the cervical spine during the third to
eighth week.
a b
c
Fi g . 2 2 . 4 A 5 5 - ye a r - o ld m a n w it h Klip p e l– Fe il s yn d r o m e a n d b a s ila r in v a g in a t i o n . Th e p a t ie n t p r e -
sented wit h severe ne ck pain and headache. (a) Lat e ral rad io g ra p h sh owin g co n ge nit al fu sio n b et we en
C3 – C4 a n d C5 –C6 . (b) Mag net ic resonance im aging also sho ws t he co nge n it ally fused verteb rae wit h
disk degeneration at C4–C5. Basilar invagination is noted as well. (c) Postoperative radiograph show-
ing an o ccip u t – C6 fu sion wit h p la t e–scre w xat ion .
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