CHAPTER
115
Surgical Treatment of
Myelomeningocele Deformities
BACKGROUND
The surgical treatment of spinal deformities associated with
myelomeningocele is intricately linked with the overall
management of the spina bifida childs health since so many
essential functions are linked to proper function and balance
of the vertebral column. Spinal deformities are pervasive
throughout the population of children with myelomeningocele
and profoundly affect the life of the child. Both congenital
and developmental deformities can exist concurrently.7 They
add further to the severity of the overall disability that is inher-
ently linked to the neurologic level of impairment. Depending
upon the level of the spinal deformity, it may bring about pul-
monary restrictions from thoracic insufficiency, inability to bal-
ance the torso in an upright posture, crowding of abdominal
viscera, and asymmetric pressure on skin leading to decubiti
(Fig. 115.1).
The lack of formation of the posterior arc in the vertebral
column is the most common defect in these children differen-
tiating them from other types of childhood spinal deformities.
In addition, the neural tube defect that brought about the
myelomeningocele has many associated anomalies throughout
the central nervous system including the Chiari II malforma-
tion (in virtually all patients), hydrocephalus (80% to 90% of
patients), as well as spinal defects that impair spinal cord func-
tion (diastematomyelia, syringomyelia, or tethered cord) (Table
115.1). The hydrocephalus has been noted to increase the rate
of skeletal maturity.5 In addition, the myelomeningocele defect
is commonly associated with other vertebral body anomalies
including congenital kyphosis and congenital scoliosis as well
as synostosis of the ribs and chest wall defects. There is also a
20% incidence of renal anomalies including absence of a kid-
ney, horseshoe kidney, and multiple anomalies of the urinary
tract system.
The three most important factors in predicting the forma-
tion of spinal deformity are the motor level, ambulatory sta-
tus, and the level of the last intact laminar arch.7 The higher
the motor deficit, the greater the likelihood of spinal defor-
mity. It is also important to keep in mind that the neurologic
level does not always correlate with the level of the last intact
arch.6
1244
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Richard E. McCarthy
GOALS
The goals of treatment of spinal deformities in myelomeningo-
cele should be the creation of a stable, well-balanced spinal
column suitable to support the torso while preventing the
development of secondary deformities. As an example, early
treatment can prevent the onset of the barrel chest deformity
seen commonly in the late development of untreated myeloky-
phosis. Early restoration of sagittal balance can prevent abdom-
inal crowding and rib flaring thus preventing what Campbell
refers to as secondary thoracic insufficiency.3
The paralytic condition is associated with lack of proprio-
ceptive feedback mechanisms required to balance the torso
and maintain sagittal balance. The persistence of pelvic obliq-
uity in the coronal plane may lead to decubiti from inability to
unweight the insensate skin over the ischium or sacrum. Timely
correction of the deformities is essential, as is the maintenance
of a growing spine to increase truncal height.
The principles of growth learned in other areas of spinal
deformities have proven successful in children with spina bifida
and the use of growing rod systems in myelomeningocele has
been shown to be efficacious.8
INDICATIONS
Coronal deformities greater than 50 warrant surgery.7 Patients
younger than 8 years should be considered for a growing
construct,8 while older patients should have a definitive fusion.
Preoperative evaluation must consider the underlying illness
and the ability of the child to withstand the physiologic demands
of extensive surgery. A preoperative magnetic resonance imag-
ing (MRI) can detect intrathecal abnormalities including teth-
ering, which is commonly associated with progressive scoliosis.
A 3-D computed axial tomography (CAT) scan may help to
plan pedicle sites for fixation and help in planning for correc-
tive osteotomies or vertebral column resections. A brain com-
puted tomography (CT) and a neurosurgical evaluation may be
necessary to evaluate the hydrocephalus. Nutritional status
needs to be considered and if lacking, a preoperative hyperali-
mentation program via nasogastric or gastric tube may be
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 Chapter 115 Surgical Treatment of Myelomeningocele Deformities
indicated. If soft tissue coverage is a concern, evaluation by
plastic surgery may determine the need for tissue expanders.1
Many, if not most, patients will require a supplementary ante-
rior lumbar fusion; rarely will anterior fusion and instrumenta-
tion alone be sufficient for correction.11
ASSOCIATED ANOMALIES
Diastematomyelia is a bony or fibrocartilaginous spur found in
the middle of the spinal canal that splits the spinal cord and
can lead to neurologic loss as the child grows, and intact neu-
rologic fibers stretch over the spur with growth. A thickened
filum terminale is the most common form of tethered spinal
cord in myelomeningocele and is found caudal to the placode.
Tethering can occur with scarring later in life, and it can lead
Associated Neural Tube
TABLE 115.1
Defects
Chiari II
Syringomyelia
Diastematomyelia
Tethered Cord
LWBK836_Ch115_p1244-1251.indd 1245
1245
Figure 115.1. Problems associated
with the upright posture in patients
with kyphosis associated with myelom-
eningocele.
to a worsening of scoliotic curves and spasticity.4 Many patients
are improved in their scoliosis and spasticity merely through
release of the tethering alone; the scoliosis most times will still
need surgical stabilization. Hydrocephalus occurs in roughly
90% of children with myelomeningocele and the majority will
need shunting. Dilatation of the central canal due to an increase
of pressure and poor flow of cerebrospinal fluid can lead to
formation of a holocord or localized syringomyelia.
The functional motor and sensory levels are related to but
not always consistent with the anatomic level of the myelom-
eningocele lesion or the last intact laminar arch. Function can
often be preserved below the anatomic levels involved in these
anomalies. Additional intrathecal abnormalities can lead to
asymmetry in neurologic function, for example, diastematomy-
elia or hemimyelomeningocele with preservation of neurologic
function in the lower extremities in an asymmetrical pattern.
SURGICAL ANATOMY OF
MYELOMENINGOCELE
The scars from the previous neurosurgical procedures can be
challenging for subsequent surgeons. Vertical midline incisions
are always preferred and the neurosurgeons carrying out the ini-
tial surgical intervention soon after birth should be encouraged
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 1246 Section X Paralytic Deformity
Figure 115.2. Scars on the back of a child with myelomeningo-
cele and kyphosis can pose a challenge in surgical planning (view
from head of patient).
to use midline incisions free of acute-angled incisions prone to
poor blood supply. Subsequent incisions for the correction of spi-
nal deformities should follow previous scars or cross these scars at
a perpendicular direction wherever possible to maximize blood
supply to the healing skin edges (Fig. 115.2). The triradiate inci-
sion has a 40% incidence of skin necrosis and if possible should
be avoided.13 Occasionally, an excess amount of skin and subcu-
taneous tissue may be present at closure that will allow for exci-
sion of the old scar. Prerequisite planning may, however, involve
the intervention of a plastic surgeon to insert tissue-expanding
devices to produce additional skin coverage for poorly covered
areas. The additional soft tissue coverage will aid in padding of
the spinal implants at the time of spinal deformity surgery.
The dura mater surrounding the neural placode lies just
under the skin and is sometimes accompanied by overlying epi-
dural fat. The dural membrane is filled with cerebrospinal fluid
and if lacerated necessitates repair (with a 4-0 nurolon on a
small tapered needle). Surgical dissection in a lateral direction
leads to the everted bony lamina, which comprises the lateral
masses evident radiographically. Spinous processes are absent
in the bifid area, and the lateral masses consist of the posterior
bone that would have migrated in a more dorsal direction. This
embryologic mesoderm was arrested in the process of posterior
migration, stopping in a lateral position unable to encircle the
neural placode as it was designed to do. The normal progres-
sion of mesodermal migration is from a ventral to dorsal direc-
tion. The accompanying paraspinal muscles that were a compo-
nent of the mesoderm were also part of the arrested migration
and were left in an anterolateral position. The paraspinal
muscles will, therefore, ironically, act as flexors rather than
TABLE 115.3 Comparison of Fixation Types
Pelvic Fixation Location of Fixation Advantage
Pelvic screws Iliac and S1 Best overall stability
S hooks Sacral ala Simple, quick
Excellent for obliquity
Galveston Iliac Strong stability
LWBK836_Ch115_p1244-1251.indd 1246
TABLE 115.2 Guidelines for Treatment
Kyphosis
Correction by age 5 yr
Continue thoracic growth
Scoliosis
8 yr Growing rods for 50
8 yr Fusion 50
extensors of the spinal column in this aberrant position. The
vertebral bodies are flattened and widened with pedicles that
converge at a more acute angle toward the midline than nor-
mal. The entrance to the pedicles lies almost horizontal in the
coronal plane. An appreciation of this convergence is critical
for the placement of pedicle screws into the vertebral bodies.
TREATMENT OF SPINAL DEFORMITY
(Tables 115.2 and 115.3)
Densely scarred and crevassed skin from previous surgeries may
need prespinal operative resection and tissue advancement to
permit better skin closure.
The actual dissection from the skin to the lateral masses
should be done in such a way as to preserve the maximum
thickness of the skin and subcutaneous tissue overlying this
area since this will be important to achieve good soft tissue cov-
erage over the spinal implants at closure (Fig. 115.3). The use
of a bifurcated incision coursing along the lateral masses join-
ing to the midline at the level of the diastasis forming an
inverted Y (the triradiate incision) is very tempting but should
be discouraged as it invariably leads to necrosis of the skin
edges at the junction of the lumbar incisions. This can be disas-
trous and lead to deep-seated infection and possibly meningi-
tis. The incidence of infection is inherently elevated in patients
with myelomeningocele due to chronic urinary tract infections
(UTIs) and, therefore, every attempt should be made to pro-
tect the vertebral structures with an intact skin closure free of
infection. Once the surgical approach reaches the lateral
masses (Fig. 115.4), the use of electrocautery will be helpful
along with a subperiosteal dissection of the lateral masses
(Fig. 115.5), freeing the paraspinal muscles from the lateral
attachment to the bony masses (Fig. 115.5C). The purpose of
this is twofold, not only to allow for approach to the bony struc-
tures for instrumentation and fusion but also to mobilize this
flap of paraspinal muscle at the completion of the surgery and
draw this muscle flap into a posterior position to enhance the
coverage. By bringing the paraspinal muscles into a dorsal posi-
tion, they are able to act as extensors of the vertebral column.
Disadvantage
Cross SI
Time to insert
Cannot control rotation
Cross SI
Time to bend and insert
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 Chapter 115 Surgical Treatment of Myelomeningocele Deformities 1247

Figure 115.3. The incision should be planned to maximize blood Figure 115.4. Digital palpation of bony prominences will reveal
supply to the wound edges for healing purposes. Blunt dissection of the lateral masses.
the tissues superficial to the dural layer should allow for maximal
thickness of skin flap.

A
B

B
Figure 115.5. (AC) Incision of the periosteum to bone of lateral
masses will permit entrance to the subperiosteal layer bilaterally to
C detach the paraspinal muscles. (A = Neuroplacode with intact dura.
C
B Lateral bony masses. C = Paraspinal muscles.)

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 1248 Section X Paralytic Deformity
Innervation of these muscles is segmental and therefore proxi-
mal innervation will be sufficient to allow these caudally
attached fibers to act as true extensors. Most myelokyphotic
patients have a bifid area that extends through the major part
of the lumbar spine; this should all be dissected free of soft tis-
sues along the lateral bony ridge at least cephalad onto that
portion of the spine where the neural arch is intact since fusion
of the bifid area is of critical importance. In addition, anterior
interbody fusion through the transforaminal lateral interbody
fusion (TLIF) approach or an additional anterior approach
may be helpful in supplying further anterior stability and
fusion. Where fusion is necessary a separate, subdiaphragmatic
anterior disc excision is best to achieve arthrodesis especially
where there is extensive lack of posterior elements, hence less
surface area for graft adherence. In young children where par-
tial preservation of growth is important, the area from the infe-
rior intact neural arch superior in a cephalad direction is the
area where growth should be preserved. The use of carefully
placed Luque wires with minimal dissection has been very help-
ful in allowing growth to proceed in a cephalad direction, espe-
cially in early correction of kyphosis (Fig. 115.6). Dissection in
the thoracic spine should be done with minimal subperiosteal
exposure and preservation of the interspinous ligament to
maximize growth. In the caudal spine, the sacral ala and iliac
wings should be dissected free of soft tissues to promote a good
fusion, which is desirable and forms a good fusion base around
the pelvic implants. Spinal reconstruction can be compatible
with the combination of lumbar stability and fusion while pre-
serving thoracic growth. This hybrid system can be used in the
treatment of both coronal and sagittal deformities.
Figure 115.6. Anteroposterior radiograph demonstrating growth
of Luque wires upward as the child grows.
LWBK836_Ch115_p1244-1251.indd 1248
Figure 115.7. Cross-sectional view of bifid vertebral with polyaxial
screw on one side and gearshift probe preparing the opposite side
tract for additional screw.
Bone fusion is dependent upon proper cleaning of the soft
tissue attachments from the posterior bony structures, proper
decortication with a burr and the use of copious amounts of
bone and bone osteogenic substances. Pseudoarthrosis has
been reported to be as high as 76%2,12 but is lowered by maxi-
mizing the fusion surfaces (anterior/posterior) and improv-
ing the fusion materials, volume, and quality, through copious
amounts of allograft and where appropriate osteogenic sup-
plements.
Pedicle screws with polyaxial heads in the bifid lumbar
spine offer the greatest versatility and accuracy in placement
(Fig. 115.7). Fixation through the thoracic spine is surgeons
choice, but pedicle screws or sublaminar wires offer the best
correction for thoracic lordosis. Fixation to the pelvis is best
accomplished with either iliac screws coupled with S1 pedicle
screws or S-hooks to the sacral ala. The choice of type for sacral
fixation is determined by the deformity being addressed.
Scoliosis is often associated with pelvic obliquity, which is read-
ily corrected with the easy to apply sacral alar S-hooks. For
more rigid obliquity or the occasional case of pelvic rotation or
severe retroversion, iliac fully threaded screws coupled with S1
pedicle screws offer extremely firm purchases to correct these
malalignments. The Galveston rod has also traditionally been
used in this setting.
The rod size should be as large a diameter as the childs size
will accommodate. Corrective osteotomies, decancellizations,
and vertebral column resections have added enormously to the
surgeons capabilities to achieve restoration of balance through
the direct correction of spinal deformities in three dimensions.
Figure 115.8 demonstrates the appearance of a bifid lumbar
spine after bilateral subtraction osteotomies and fixation via
pedicle screws at the levels above and below the osteotomies.
Compression is being applied to bring the posterior elements
together and correct the kyphotic deformity. The correction
can be firmly stabilized by dual rods and pedicle fixation.
Secure pelvic fixation can supply a strong foundation upon
which to base the correction. Figure 115.10 shows an example
of this correction while Figure 115.9 demonstrates use of a
growing rod construct.
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 Chapter 115 Surgical Treatment of Myelomeningocele Deformities
Figure 115.8. After placement of pedicle screws, the bilateral
pedicle subtraction osteotomies allow the curvature to be corrected
and held with a provisional rod.
A B
D E
LWBK836_Ch115_p1244-1251.indd 1249
1249
GROWING RODS IN
MYELOMENINGOCELE
Growing rod constructs have been used in a limited number of
patients with myelomeningocele. The application in a patient
population such as this, which is known to characteristically
reach maturity at an earlier age than most patients of compa-
rable age is somewhat controversial. The importance of main-
taining maximum truncal height in a sitting patient is paramount
due to the encroachment of the thoracic cavity from the
abdominal pressure elevating the diaphragm in the sitting pos-
ture. Compromised sitting height from early spinal fusion or
spinal deformity can lead to thoracic insufficiency and early
demise.3,10
Fifteen patients have been reported8 with greater than 2-year
follow-up (average 6 years) using growing rod techniques. A
variety of surgical techniques were used including single-rod,
dual-rod, vertical expandable prosthetic titanium rib (VEPTR),
and hybrid VEPTR techniques with good results. The space
available for the lung and truncal height were improved 12%
C
Figure 115.9. Immature myelodysplastic chil-
dren with severe scoliosis can be treated with
growing rods. (A) Preoperative upright poster-
oanterior (PA) radiograph of a 7-year-old girl
with 92 of scoliosis and severe restrictive pulmo-
nary compromise. (B) Upright lateral preopera-
tive radiograph. She was placed in halo traction
at home for 2 months with an in traction film
(C) and postoperative PA and lateral radiographs
(D and E) after placement of a Shilla growing rod
construct.
8/29/11 9:00:12 PM
 1250 Section X Paralytic Deformity

A B C

D E F

Figure 115.10. A 12-year-old T10 level myelodysplastic with 146 of scoliosis, apex T8, and 90 kyphosis
with apex L2 (A and B). The procedure was a one-stage posterior only vertebral column resection of T8 and
T9 with a pedicle subtraction osteotomy at L2 with (CF) the postoperative radiological and clinical results.

with 50% improvement in curve magnitude at follow-up. The
Shilla growth guidance technique has been used in four
patients, also with good results at shorter follow-up but without
the need for repeated operative lengthening every 6 months as
required by other techniques. One patient required rod
removal due to prominent implants. The complication rate in
this patient population has been surprisingly low for all types of
growing rods and comparable to other groups undergoing
growing rod treatment. Rod breakage due to metal fatigue was
seen in three patients and the only infection occurred in the
sole patient with implant prominence and skin breakdown
mentioned above.
With this limited experience, we feel secure in recommend-
ing the use of growing rod systems in young children (less than
8 years of age) with severe deformities rather than early spinal
fusion with the resultant loss of truncal height and compromise
in pulmonary capacity.
CONCLUSION
Few long-term studies have been reported in the treatment of
spinal deformities in myelomeningocele. Niall et al9 reported a
high complication in children undergoing kyphectomy with
skin problems predominating; this has been borne out by oth-
ers. The effort, however, to correct these deformities is worth;
the effort is to (1) maintain an upright posture and restore a
semblance of normality, (2) return them to their families and
their communities to become productive citizens, and (3) to
minimize the burden of their disability on society. As we

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 Chapter 115 Surgical Treatment of Myelomeningocele Deformities 1251

proceed forward, quality of life assessments can be developed
that take into consideration the varied level of neurologic
involvement of these patients while assessing the secondary
effects on respiration and physical activity in these children.
5. Feeley BT, Ip TC, Otsuka NY. Skeletal maturity in myelomeningocele. J Pediatr Orthop
2003;23:718721.
6. Glard Y, Launay F, Hamel A, et al. Neurological classification in myelomeningocele as a
spine deformity predictor. J Pediatr Orthop B 2007;16:287292.
7. Guille JT, Sarwark JF, Sherk HH, et al. Congenital and developmental deformities of the
spine in children with myelomeningocele. J Am Acad Orthop Surg 2006;14:294302.
8. McCarthy RE, Muharrem Y, Akbarnia BA, et al. Growing rods in myelomeningocele for
scoliosis treatment. International Meeting Advanced Spine Techniques Annual Meeting;

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9. Niall DM, Dowling FE, Fogarty EE, et al. Kyphectomy in children with myelomeningocele:
a long-term outcome study. J Pediatr Orthop 2004;24:3744.
1. Arnell K. Primary and secondary tissue expansion gives high quality skin and subcutaneous
10. Pehrsson K, Nachemson A, Olofson J, et al. Respiratory failure in scoliosis and other tho-
coverage in children with a large myelomeningocele and kyphosis. Acta Neurochir
racic deformities. Spine 1992;17:714718.
2006;148:293297.
11. Sponseller PD, Young AT, Sarwark JF, et al. Anterior only fusion for scoliosis in patients
2. Banit DM, Iwinski HJ, Talwalkar V, et al. Posterior spinal fusion in paralytic scoliosis and
with myelomeningocele. Clin Orthop Relat Res 1999;364:117124.
myelomeningocele. J Pediatr Orthop 2001;21:117125.
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3. Campbell RM Jr, Smith MD, Mayer TC, et al. The characteristics of thoracic insufficiency
eningocele. Eur J Pediatr Surg 1998;8:2225.
syndrome associated with fused ribs and congenital scoliosis. J Bone Joint Surg Am
13. Ward WT, Wenger DR, Roach JW. Surgical correction of myelomeningocele scoliosis: a
2003;85A:399408.
critical appraisal of various spinal instrumentation systems. J Pediatr Orthop 1989;9:
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