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21 Pediat ric Spinal Deform ity 237
a bcd
ef gh
ij
Fi g . 2 1 . 5 ( a – j) Ve r t i c a l e x p a n d a b l e p r o s t h e t i c t i t a n i u m r i b i n t h e p e d i a t r i c s p i n a l d e f o r m i t y p a t i e n t .
238
21 Pediat ric Spinal Deform ity
Fig . 2 1 . 6 Ty p e s o f c o n g e n i t a l
scoliosis. (a) Unilateral unsegmented bar. (b) Block vertebra.
(c) Fully segmented hemivertebra. (d) Semisegmented hemivert eb ra. (e) Nonsegmented
hemivertebra.
a
b
c d e
C. De p e n d i n g o n i n s t r u m e n t at io n u s e d , co r re ct io n p e r ce n t ag e va r ie s fr om 5 0 t o
75%.
D. Fu sion below L3 in creases t he in cid e n ce of low e r b a ck p ain .
E. Po st e r ior sp in al in st r u m en t a t io n h as a r e o p e r a t io n r at e o f 5 t o 1 9 %.
F. Ot h e r c o m p l i c a t i o n s :
1. Delayed infection.
a. Incidence of 1 to 7%.
b. Requires hardware removal and antibiotics.
2. Late-onset surgical pain.
a. Incidence of 5%.
b. Requires hardware removal.
3. Pseudarthrosis.
a. Incidence of 3%.
b. Compression instrumentation or bone graft needed for treatment.
21 Pediat ric Spinal Deform ity 239
21.3 Idiopathic Infantile and Juvenile Scoliosis
I. Id io p at h ic in fan t ile scoliosis.
A. Us u ally d e t e ct e d at 2 t o 3 m on t h s o f a ge .
B. Gr e at er in cid en ce in b oys t h a n g i r ls .
C. Mo r e co m m o n in En gla n d .
D. Nin et y p ercen t o f cases involve th e left t hora cic.
E. Pr o gn os is:
1. Sixty to 70% of cases resolve spontaneously.
F. Tw o t y p e s o f p r o g r e s s i v e c u r v e s :
1. Benign curves are typically characterized by > 1-year onset, double curves,
and greater exibility.
2. Malignant curves are characterized by > 1-year onset, thoracic location, and
rigid curves.
G. Good p rogn osis if Meh t a angle (rib –vertebral angle) is < 20° an d the convex rib
does not overlap the vertebral body on the posteroanterior radiograph (phase I).
In p h a se II, t h e r e is a n ove rla p bet w ee n t h e r ib a n d ver teb r a l b o d y, an d t h e
prognosis is worse.
H. Th e t reat m en t con sist s of bracing for cu rves > 30°. If the cur ve is p rogressive,
surgery is recommended. Options include subcutaneous rod or telescoping rod
without fusion or combined anterior and posterior fusion.
II. Id io p at h ic ju ve n ile s co lio s is.
A. Th e r igh t t h or acic p at te r n is t h e m o s t co m m on .
B. Va r ia b le p ro gr e s s io n : o n e- t h ir d a re o b se r ve d , o n e - t h ir d a re b ra ce d , a n d o n e -
third require surgery.
C. Brace if > 30°.
D. Su rger y if progressive cur ve > 45° desp ite brace t reatm en t , especially du r ing
puberty.
21.4 Other Type of Scoliosis
I. Co n ge n it al s co lio sis (Fig. 21.6).
A. Fa ilu re o f s egm e n t at io n o r fo r m at io n o r b ot h .
B. As s ocia t e d a n o m a lie s: ge n it ou r i n a r y (r en a l a g e n e s is , u re te ra l o b s t r u ct io n ) .
C. Pa ra ly t ic s co lio si s .
D. Neu ro brom atosis.
II. Ne u r o m uscu lar scoliosis (Fig. 21.7).
A. Ge n e r a l co n sid e r a t io n s .
1. Bracing does not prevent the natural progression of the scoliosis.
2. Di erences in surgical approach include the length of fusions and operating
on sm aller curves.
3. Hooks and screws are used more frequently.
4. Segmental Luque wires are used.
5. Increased rates of complications.
B. Ce r e br a l p als y.
1. Scoliosis a ected by the imbalance of the paraspinal muscles.
240
21 Pediat ric Spinal Deform ity
ab
cd
Fi g . 2 1 . 7 A 1 3 - ye a r - o ld b o y w it h n e u r o m u s c u la r s c o lio s is . (a) Anteroposterior and (b) lateral radio-
graphs. There appears to be lumbar hyperlordosis and a compensatory thoracic kyphosis. (c,d) Po st o p -
erative radiographs demonstrate a combination of pedicle screw, hook, and sublaminar wiring xation.
21 Pediat ric Spinal Deform ity 241
2. Surgery.
a. Cu rves > 50 °.
b. Levels to be repaired:
(1) Am bulatory patient.
(a) Proximal to distal stable vertebra.
(2) Nonam bulatory patients.
(a) T2 to pelvis.
c. Usually posterior approach but anterior approach may need to be
perform ed for cur ves > 100°.
C. M ye lo m e n in go cele .
1. Secondary to birth defect: exposure of the meninges and spinal cord may
result in bowel, bladder, motor, and sensory de cits.
2. Incidence of 1 in 1,000: correlated with folate de ciency in pregnancy.
3. Fifteen percent of patients with this de cit are allergic to latex.
4. MRI is used for diagnosis due to the neurological complications.
5. Surgery:
a. Indicated for patients who have di culty with sitting or are at risk for
developing pressure sores.
b. A combined posterior and anterior approach is used.
D. Spin al m u scu lar at rop h y.
1. Progressive weakness due to loss of anterior horn cell neurons.
2. Three types.
a. Type I (Werdnig–Ho mann disease).
(1) Onset in neonatal period and death by age 2.
b. Type II.
(1) Onset at ~ 5 to 6 months of age.
c. Type III.
(1) Onset before age 3 and progressive weakness with loss of am bulatory
strength by age 15.
(2) Surgery.
(a) Indicated in progressive scoliosis.
(b) The approach should be an terior and posterior for a young
patient with large curves and only posterior for the older patient
with smaller curves.
E. Du ch e n n e ’s m u sc u la r d yst r o p h y.
1. X-linked recessive disorder.
2. Spinal deformity develops secondary to muscle imbalance only after loss of
ambulation.
3. Increased incidence of malignant hypertension with general anesthesia.
4. Preoperative pulmonary function and cardiac contractility assessment are
necessary.
5. Surgery:
a. Indicated in progressive scoliosis > 25 to 30%.
b. A T2 to sacrum posterior approach is used.
242
21 Pediat ric Spinal Deform ity
21.5 Kyphotic Deformities
I. Ge n e r a l con sid e r at ion s of k yp h ot ic d e fo r m it ie s.
A. Ana t o m ic a l var iat io n .
1. Cervical lordosis, thoracic kyphosis, and lumbosacral lordosis
2. The sagittal plumb line (odontoid) should normally cross C7–T1, T12–L1,
and the posterior aspect of the sacrum (S1).
3. Normal thoracic kyphosis:
a. Tw e n t y to 45°, m ean = 34°.
4. Normal lumbar lordosis:
a. For t y to 60°.
b. Two-thirds of lordosis occur between L4–L5 and L5–S1.
B. Bio m e ch a n i c s .
1. Anterior column failure results in compression, and posterior column failure
results in tension.
2. Posterior structures:
a. The lamina and ligamentum avum are relatively stronger in resisting
tension than facets, capsules, and interspinous ligaments.
3. Deformity increases the moment arm, which can result in further
decompensation.
4. Eccentric loading can a ect cartilaginous growth.
a. Compression decreases growth anteriorly.
b. Tension increases growth posteriorly, resulting in increased kyphosis.
C. Cla ss i c a t io n (Table 21.4).
D. Speci c k yph ot ic d efor m it ies.
1. Postural kyphosis.
a. Most common in adolescents and young adults.
b. Round back deformity.
c. Mod est kyph osis (40– 60°).
d. Smooth and exible.
e. No radiographic changes.
f. No sp eci c t re at m e n t —co n se r vat ive m an age m en t .
2. Congenital kyphosis.
a. May be single or multilevel.
(1) Type I.
(a) Failure of form ation (hem ivertebra).
(b) Worse progn osis for progression an d paraplegia.
(c) Upper spinal levels have a worse prognosis than lower levels.
(2) Type II.
(a) Failure of segm entation (bar).
(3) Type III.
(a) Com bination of I and II.
(4) Treatm ent.
(a) Nonoperative treatment is ine ective.
Ta b l e 2 1 . 4 Classi ca t ion o f k yp h o t ic d efo rm it ie s
Ty p e N o t e s
Post u ra l
Co ng en it a l De fe ct o f fo rm at io n
Defect o f se gm e nt at io n
Mixe d
Neuro m u scu lar (Fig. 21.8)
Sch e ue rm an n ’s d ise a se (Fig. 21.9)
21 Pediat ric Spinal Deform ity 243
Mye lo m e nin g o ce le De ve lo pm e n t a l (lat e p ara lyt ic)
Co ng e nit a l (p re se n t a t b ir t h )
Tr a u m a t i c Due t o bo n e , ligam e n t , and / or cord injury
Post su rgica l Post la m in ect o m y
Fo llo w in g e xc is io n o f a ve r t e b r a l b o d y
Post irradiat io n
Me t ab olic Ost e op o ro sis
– Senile
– Juvenile
Osteom alacia
Osteogenesis imperfect a
Ske let al d ysp lasia Ach o nd rop lasia
Muco p olysacch arid o s e s
Neuro brom ato sis
Co lla ge n d is e ase Ma rie –St rü m p e ll d ise as e
Tu m o r Be n i g n
Malig n an t
– Primary
– Metastatic
In a m m a t o r y a nd in fe ct io us
(b) Surgery:
i. Type I lesion s.
• Posterior in sit u fusion if < 50 ° at 1 to 5 years.
• Anterior an d p osterior fusion if > 50° an d old e r ch ild ren.
▫ Better correction and maintenance.
▫ Lower rate of pseudarthrosis.
244
21 Pediat ric Spinal Deform ity
• An te r ior decom pression .
▫ Release of all the tethering structures:
– Anterior longitudinal ligament.
– Intervertebral disk and end plate.
– Posterior longitudinal ligament.
▫ Intraoperative distraction and correction of the deformity:
– Rib, bula, or iliac crest strut grafts.
▫ Simultaneous or second-stage posterior fusion with
compression instru m entat ion .
▫ Postoperative bracing is recommended.
ii. Type II lesion s.
• Posterior fusion on ly if kyp h osis is < 55°.
▫ For severe deformity, an anterior osteotomy and
correction and fusion m ay be at tem pted follow ed by a
posterior fusion.
• Skelet a l t ra ct ion is con t ra in dicat e d as it m ay cau se
paraplegia.
3. Scheuermann’s disease (juvenile kyphosis).
a. Scheuermann rst described radiological manifestation of this disease in
1920.
(1) The incidence is 0.4 to 8.3% of the population, but only 1% seek
medical attention.
(2) Pathogenesis is unknown.
(a) Fam ilial tendency with no genetic link.
(b) Collagen weakness and stunted ossi cation of th e vertebral end
plate are characteristic.
(c) Osteopenia.
(d) Nutritional de ciencies.
(e) Mechanical alterations and muscle weakness have been
theorized.
i. No scien t i c evid ence exist s.
ii. Grow th centers adjacen t to th e vertebral end plate (n ot ring
apophyses):
• An ter ior car t ilagin ou s colu m n s exp erien ce st un te d grow th
with axial loading.
• Poster ior p hysis h yper t rophy d ue t o t e n sile forces.
iii. With kyphotic deform ity, spinal exors becom e st ronger th an
extensors because of the m oment arm.
(3) Pathoanatomy:
(a) Thickened and contracted anterior longitudinal ligam ent.
(b) Wedging of the anterior vertebral bodies.
(c) Nucleus pulposus.
i. Protrusion an teriorly an d into the bony spongiosa (Schm orl’s
nodes).
(4) Clinical ndings:
(a) Onset is com m only between 12 and 14 years of age.
(b) Equ al male:fem ale ratio.
(c) Deform ity is the most com mon presenting complaint.
(d) Pain occurs in ~ 50% am ong those who seek medical attention.
i. Increased rate of symptom atology if the lum bar spin e is involved
• Som e pat ien ts d evelop lu m b ar sp on d ylolysis later.
(5) Physical exam ination:
(a) Increase in thoracic kyphosis (rigid).
(b) Com pen satory lum bar an d cervical lordosis:
i. Roun d sh ou lders.
ii. Forward tilt ing of the head.
(c) Muscle tightness and contractures are seen, typically in the ham strings.
(d) Thirty percent have an associated mild scoliosis.
21 Pediat ric Spinal Deform ity 245
(6) Radiographic ndings:
(a) Early:
i. Disordered endoch on dral ossi cat ion .
ii. Irregular en d plates.
iii. Narrow ing of t h e interver tebral disk space.
iv. Sch m orl’s nodes.
(b) Interm ediate:
i. Vertebral wedging.
ii. In creasing kyph osis > 45°.
• More t h an 5 ° of an te r ior w e dgin g in t h ree or m ore
vertebrae at the ap ex of th e kyphosis (Sorenson’s criteria).
(c) Late:
i. Degenerative changes.
• Osteop h yt es.
• Facet hyp e r t rop h y.
(d) Standing lateral and supine hyperextension views are used to
assess the rigidity of the curve.
(7) Treatment:
(a) Observation is indicated for mild deform ity with minim al
symptoms.
(b) Bracing indications:
i. Ver t ebral w edgin g > 5°.
ii. Kyph ot ic cu r ves bet ween 45 an d 65° and 1 to 2 years of
grow th rem aining:
• Milw aukee brace for apex above T9.
• Thoracolu m bar Sacral Orth osis (TLSO) for th e ap ex b elow
T9 a n d t h o ra co lu m ba r cu r ve s.
iii. Curve correction and wedging im p rovem en t of ~ 40% can be
expected after 6 to 12 months.
iv. The brace sh ould be w ean e d w it h skeletal m aturit y, but loss of
correction is expected after 10 years.
(c) Exercise:
i. Stressin g pelvic tilt , abdom in al strengthening, spinal
exibility, and thoracic spine extension exercises is an
important part of the treatm ent plan.
246
21 Pediat ric Spinal Deform ity
(8) Surgery:
(a) Indications.
i. Severe deform it y after grow th com plet ion w ith unrelenting
pain.
• Typ ically > 75° an d > 10° w edging in three or m ore
cont iguous vertebral bodies.
ii. Resistan ce to bracing after 6 m on ths.
iii. Neurological signs or sym ptom s.
(b) Techniques.
i. Posterior-on ly instrum ented fusion .
• Cur ves < 75° and ben din g cor rect ion to < 50°.
• Post e r ior in str u m en t ation sh ou ld ext e n d th e e n tire
kyphotic region, and distally it should include one lordotic
vertebra (usually L1 or L2). The posterior sacral vertical
line is a vertical line th at crosses the posterior su perior
corner of the sacrum . The vertebra that bisects this line
is som etim es con sidered as the end vertebra for fusion to
prevent junctional deformity.
ii. An terior fusion (transth oracic approach) followed by posterior
fusion an d in st rum en tat ion.
• Cur ves > 75° w it h m in im al bendin g cor rection (> 50°).
(c) Postoperative protocol.
i. TLSO for 6 to 9 m on ths un til solid fusion.
(d) Complications.
i. Pseudarthrosis and instrum entat ion failure (greater in
posterior fusion alone).
ii. Loss of correction.
iii. Infection.
iv. Pulm onary com plicat ion s.
v. Ne u rologica l de cit s.
4. Neuromuscular kyphosis (Fig. 21.8).
a. Associated conditions:
(1) Poliomyelitis.
(2) Anterior horn cell diseases (spinal muscular atrophy).
(3) Cerebral palsy.
(4) Charcot–Marie–Tooth disease.
(5) Muscular dystrophy.
(6) Friedreich’s ataxia.
b. Lack of the extensor muscle strength contributes to development of a
kyphotic deformity.
c. The natural history is progressive even after skeletal m aturity.
d. Treatment:
(1) Bracing until the patient is ~ 11 to 12 years old to maximize truncal
height.
(2) Posterior fusion with instrum entation in milder and exible curves.
(a) Com pression instrum entation is preferred over Luque rods with
sublaminar wires for correction of kyphosis.
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