J Child Orthop (2009) 3:441–449
DOI 10.1007/s11832-009-0214-5

CURRENT CONCEPT REVIEW

Orthopedic management of spina bifida. Part I: hip, knee,
and rotational deformities
Vineeta T. Swaroop • Luciano Dias

Received: 20 August 2009 / Accepted: 3 October 2009 / Published online: 25 October 2009
Ó EPOS 2009

Abstract Children with spina bifida develop a wide
variety of congenital and acquired orthopedic deformities.
Among these are hip deformities such as contracture,subluxation, or dislocation. Patients may also have problems with the knee joint, such as knee flexion or extension
contracture, knee valgus deformity, or late knee instability
and pain. In addition, rotational deformities of the lower
extremities, either internal or external torsion, are common
as well. This paper will review both the overall orthopediccare of a patient with spina bifida and provide a focused
review of the diagnosis and management of the above
deformities. In addition, this paper will review the incidence, etiology, classification, and prognosis of spina
bifida. The use of gait analysis and orthoses will be covered
as well. The forthcoming Part II will cover foot and ankle
deformities in spina bifida.
Keywords Spina bifida  Myelomeningocele 
Hip deformity  Knee valgus  Rotational deformity

Introduction
Neural tube defects (NTDs) result from failure of theneural tube to close during embryogenesis and are thecause of chronic disability of between 70,000 and 100,000
individuals in the United States [1]. Although the incidence
V. T. Swaroop (&)  L. Dias
Orthopedic Surgery, Northwestern University Feinberg Schoolof Medicine, 345 E. Superior, #1132, Chicago, IL 60611, USAe-mail: vswaroop@ric.org
L. DiasMotion Analysis Center, Children’s Memorial Hospital,
Chicago, IL, USA

of NTDs has declined in recent decades, spina bifida (alsoknown as myelomeningocele) remains the most common
NTD and is the most severely disabling birth defect compatible with survival [1].
Spina bifida is a myelodysplasia of the neural elements
which manifests in the vertebrae as a defect in the posterior
elements. Dysplasia of the spinal cord and nerve roots leads
to bowel, bladder, motor, and sensory paralysis below thelevel of the lesion [2]. Patients with spina bifida may often
have other associated spinal cord lesions, such as diastematomyelia or hydromyelia, or structural abnormalities ofthe brain, such as hydrocephalus, which may also compromise neurological function [2].
Both congenital and acquired orthopedic deformities areseen in patients with spina bifida. Examples of congenital
deformities, which are present at birth, are kyphosis,
hemivertebrae, teratologic hip dislocation, clubfoot, andvertical talus. Acquired developmental deformities are
related to the level of involvement [3] and are caused bymuscle imbalance, paralysis, and decreased sensation in thelower extremities [4]. Additionally, orthopedic problems
may also be caused by iatrogenic injury such as postoperative tethered cord.

Incidence
The incidence of infants born with NTDs shows regionalvariations but is decreasing overall. The birth–prevalence
rate of spina bifida from 1983 to 1990 in the United States
was 4.6 per 10,000 [3]. Since that time, there has been adecrease in the number of new cases of spina bifida. Thisdecrease can be attributed to two main factors: prenatal
screening with elective termination of affected pregnancies
and increased awareness of the importance of

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administration of folate to women before and during
pregnancy, as recommended by the US Public HealthService.
An estimated 50–70% of NTDs can be prevented
through the daily consumption of 400 lg of folic acid [5].
The US Food and Drug Administration mandated adding
folic acid to all enriched grain products by January 1998
[6]. From October 1998 to December 1999, the birth–prevalence rate of spina bifida in the Unites States
decreased 22.9% compared with 1995–1996 [6]. Furthermore, comparing the incidence during the period from1999 to 2000 to the period from 2003 to 2005, the prevalence of spina bifida in the United States decreased 6.9%,
from 2.04 to 1.90 per 10,000 live births [6].
In a population-based study examining the effect of folicacid supplementation on the prevalence of NTDs in 16
European countries, a 32% decrease in NTDs was foundwhen comparing the periods from 1989–1991 and 1999–
2001 in the United Kingdom and Ireland, where supplementation was introduced in the early 1990s [7]. A reduction in prevalence of NTDs of 17% was found in countries
with folic acid supplementation introduced by 1999. In
contrast, a decrease of 9% was seen in countries with nosupplementation policy by 1999.

Etiology
Spina bifida results from failure of the neural tube toclose during the fourth week of embryogenesis. The
cause of this embryonic failure is not known but issuspected to be multifactorial in origin, involving both
genetic and environmental factors. Folate deficiency is an
important contributor to the cause of NTDs as evidenced
by the decrease in incidence observed after folate supplementation. Other environmental factors have also been
examined for a potential role in NTDs, including temperature, drug exposure, substance abuse, maternal
infection, and other nutritional factors, such as vitamin
B12 and zinc [8].
Genetic factors seem to play an important role in thedevelopment of spina bifida. Association with single genedefects, increased recurrence risk among siblings, and ahigher frequency in twins than in singletons indicate agenetic contribution to the etiology [8]. A recent study
investigated the role of cell adhesion molecules which are
involved in cell–cell interactions as a possible factor in theformation and closure of the neural tube [9]. Animalstudies have shown as many as 100 mutant genes which
affect neurulation, and almost all have homologs in humans[8]. However, the low frequency of families with a significant number of NTD cases makes research into genetic
causation difficult.

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Overview of orthopedic care
The overall care of children with spina bifida has changed
substantially over the past 30 years with regards to all
specialties, including neurosurgery, urology, rehabilitation,orthotics, and orthopedics. In the realm of orthopedics,
these changes have occurred as a result of a better understanding of deformities and their affect on function. The
advent of gait analysis in the late 1980s has played a majorrole in shifting the focus of orthopedic treatment fromradiological changes to functional improvement [10].
The main goal of orthopedic care of a patient with spina
bifida is to correct deformities that may prevent the patientfrom using orthoses to ambulate during childhood [2]. In
addition, the orthopedic surgeon must monitor spinal balance and deformity and assist in monitoring the neurologic
status. The newborn examination should include identification of the level of paralysis for each extremity, as wellas identify associated problems such as clubfoot or hip or
knee contractures. The follow-up periodic orthopedic
examination should include assessment of motor and sensory function, range of motion, spinal deformity, andintegrity of skin. Changes should alert the physician to thepossibility of tethered cord. In addition, mobility andbracing needs should be addressed to ensure that orthoses
are appropriate, in good shape, and not causing any pressure points on the skin.
Orthopedic care of the child with spina bifida is madechallenging by the presence of multiple medical comorbidities which must be taken into account in any treatment
plan. These comorbidities include central nervous system
involvement such as hydrocephalus, syringomyelia, andtethering, insensate skin, latex allergy, renal anomalies, andbowel and bladder incontinence [3]. In addition, patientswith spina bifida may have precocious puberty, cognitive
learning difficulties, or depression [2]. For this reason,
whenever possible, orthopedic care should be rendered aspart of a multi-disciplinary team, working together withneurosurgery, urology, and physiatry.
Providers should also be aware that patients with spina
bifida are at increased risk for certain complications, hence,
special precautions may be indicated. Complications in thispopulation may relate to latex allergy or increased risk of
post-operative infection. Infection of the urinary tract may
result from bladder paralysis, and wound infection can
result from the lack of protective pain sensation. In addition, special care must be taken to avoid pressure sores.
Patients with spina bifida are also at increased risk forpathologic fractures due to joint contracture and post-surgical immobilization, especially spica casting. Pathologic
fractures are more prevalent in patients with a higher levelof neurologic involvement due to the presence of osteopenia related to their relative lack of mobility [4].

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Prognosis for ambulation
Attaining early ambulation can provide physiological andpsychological benefits to a child with spina bifida, even ifthat child will later become a sitter. A study comparing
patients with high-level spina bifida who had participatedin a walking program with those who had been prescribed awheelchair early in life found patients who walked early
had fewer fractures and pressure sores, were more independent, and were better able to transfer [11]. Of note, not
all orthopedic surgeons support this idea, and anotherschool of thought does exist which disputes the benefits ofearly ambulation.
Various factors affect the potential for ambulation in an
individual patient with spina bifida. Among these areneurologic level of involvement, hip deformity, scoliosis,
foot and ankle deformity, age, and obesity [12]. Multiple
studies have demonstrated the crucial role that neurologic
level of involvement and resulting muscle group strength
plays in achieving and maintaining ambulation. In a studyof 98 patients aged 5–31 years, 20 of 21 patients with L5 orsacral level of involvement were community ambulators[12]. In the same study, most L4 patients were also community ambulators, but patients with L3 or above level ofinvolvement were mostly non-functional ambulators.
Similarly, a review of 29 adult spina bifida patients aged
20–43 years found that 19 of 20 patients with L3 or lower
level of involvement were ambulatory, while only 2 of 9
patients with L2 or above level of involvement remained
ambulatory [13]. In this study, the status of the hip did notcorrelate with the ability to ambulate.
One of the most important physical factors for maintaining ambulation in adulthood is the strength of thequadriceps and hamstrings muscles [2, 12, 14]. In a studyof 109 patients, quadriceps strength correlated strongly
with ambulatory ability. Eighty-two percent of patientswith grade 4 or 5 quadriceps power became community
ambulators, and 98% were at least household ambulators[15]. Including grade 3 power, 89% were at least household
ambulators. In contrast, 88% of patients with grade 0, 1, or
2 quadriceps power were not functional ambulators. Iliopsoas strength has also been shown to play an importantrole in ambulation. In a study of 291 patients with averageage of 14.5 years at the last examination, 77 of 87 patientswith iliopsoas strength grade 3 or less were non-ambulatory[16]. In the same study, 163 patients with symmetrical
grade 4 or 5 iliopsoas strength were all ambulatory. In
addition, one study found that achieving sitting balance
was an important predictor of ambulatory potential inpatients with higher levels of involvement [17].

Classification
The most widely used classification of spina bifida is basedon the neurologic level of the lesion [17, 18] (see Table 1).
Patients can be divided into three groups based on lesion
level and accompanying functional and ambulatory
capacity. Group 1 consists of thoracic and high-lumbar
level patients. The functional hallmark of this group is thelack of quadriceps function. Consequently, in order toambulate children requires orthoses which span the hip,and the majority of patients require a wheelchair formobility in adulthood.
Group 2 patients have low-lumbar level of involvement.
These patients retain quadriceps and medial hamstring
function but lack function of the gluteus medius andmaximus. Patients in this group require crutches and braces
to control foot and ankle position in order to ambulate.
Approximately 80% of the patients in this group maintaincommunity ambulation as adults [17]. Since medial hamstring function is needed for community ambulation, there

Table 1 Spina bifida classificationGroup

Level of lesion

Functional hallmark

Ambulatory capacity

FMS classification

1

Thoracic, high-lumbar

Lack quadriceps function

As children, require hip-spanning orthosis
for ambulation (RGO, HKAFO)FMS 1,1,1

In adulthood, majority require wheelchair
for mobility
2

3

Low-lumbar

Lack gluteus medius and maximus
function

Require crutches and AFOs
for ambulation

Retain quadriceps and medial
hamstring function

Most retain community
ambulation as adults

SacralRetain quadriceps and gluteus
medius function

High-sacral

Lack gastrocnemius-soleus
function

Ambulate with AFOs and no support

Low-sacral

Retain gastrocnemius-soleus
function

Ambulate without braces or support

FMS 3,3,1

FMS 6,6,6

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is a notable difference in ambulatory ability between
children with L4 and L3 level lesions [12]. For this reason,children with L4 level of involvement have the mostpotential benefit from proper orthopedic care of musculoskeletal deformities [18].
Patients in Group 3 have sacral level of involvement anddemonstrate both quadriceps and gluteus medius function.
Group 3 can be further subdivided into high-sacral andlow-sacral level of involvement, distinguished by thepresence of gastrocnemius-soleus strength in low-sacral
patients. Patients with high-sacral level walk without support but with the use of braces for the foot and ankle. These
children have a characteristic gluteus lurch during gait.
Low-sacral level patients walk without braces with a gaitthat is close to normal.

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rotational abnormalities [21–25]. Clinical evaluation ofpatients with spina bifida is complex and involves multiple
inter-related levels of deformity. Often, a patient’s true
functional status is different from what would be expectedbased on information obtained during the static clinicalexamination [23]. This was demonstrated in a study
examining crouched gait in patients with spina bifida [23].
The authors found a significantly greater amount ofdynamic knee flexion during ambulation using gait analysis
than what was measured on clinical examination. Computerized gait analysis should be employed as an importantpart of the examination of patients with spina bifida,
especially when surgical treatment is being considered.

Orthoses
Functional Mobility Scale
The Functional Mobility Scale (FMS) was initiallydescribed in 2004 as a useful tool to describe functional
mobility in children with cerebral palsy [19]. Recently, it
has also been applied to children with spina bifida [20].
The FMS is unique because it allows quick, practicalscoring of mobility over three distinct distances representing mobility in the home (5 m), at school (50 m), andin the community (500 m). In this way, it is effective for
distinguishing between groups of children with varying
levels of disabilities and provides a means for standardized
communication between health professionals [19].
For each of the three distances assessed, a child is given ascore from one to six based on their walking ability. A scoreof one is used when a child uses a wheelchair, two for awalker, three for the use of two crutches, four for the use of
one crutch, five for a child who is independent on levelsurfaces, and six for a child who is independent on all surfaces. Two additional ratings used are C for a child who
crawls for mobility in the home and N for a child who does
not complete the given distance. For example, a child who
uses a wheelchair for long distances but ambulates with
crutches at home and school would be an FMS 3,3,1(see Table 1).

Gait analysis
Computerized gait analysis is a valuable component of thecomprehensive orthopedic evaluation of patients with spina
bifida. It is especially useful in pre-operative planning forambulatory patients and in quantitatively assessing surgicalresults. Its use has been reported in the literature for analyzing many manifestations of spina bifida, including hip
subluxation/ dislocation, lower extremity contractures, and123

Almost all children with spina bifida, with the exception ofsome patients with low-sacral level involvement, will
require the use of orthoses for ambulation. There are many
indications for the use of orthoses in the management ofchildren with spina bifida, including maintenance ofalignment, prevention of deformity, correction of flexible
deformity, facilitation of independent mobility, and protection for the insensate limb.
Children with thoracic and high-lumbar level involvement
often have hip flexor and adductor function, but not quadriceps function, hence, orthoses are needed for upright weightbearing and mobility. This begins with a standing frame,
often prescribed around 12 months of age or once the childdemonstrates development of head and neck control. Patients
will then require an orthosis which crosses and controls the
hip in order to control the trunk over the pelvis and lower
limbs, such as a hip–knee–ankle–foot orthosis (HKAFO) or
reciprocating gait orthosis (RGO) (Fig. 1). The RGO is
usually introduced around 24 months of age. An importantfactor in the ability to walk with an RGO is sitting balance
without hand support. If a child is unable to do this, he or she
will be better served with a parapodium. It is important forproviders to recognize that most patients with higher levels of
involvement will eventually choose a wheelchair as a moreenergy-efficient way to achieve mobility [2].
Patients with sacral or low-lumbar level involvement
require a solid ankle–foot orthosis (AFO) to address themuscle weakness present below the knee [24]. The solid
AFO substitutes for weak or absent ankle plantar flexors
and dorsiflexors. Because these patients also have weakness of hip extensors and abductors, the use of forearm
crutches should be considered to improve pelvic and hip
kinematics. Crutches allow the upper extremities to sharein weight-bearing, hence, decreasing the demand on thelower extremity musculature, allowing a more functional
gait pattern [24].

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Fig. 1 a Reciprocating gait orthosis (RGO), frontal view. b RGO,
side view

Patients with rotational deformities, either internal orexternal, will benefit from AFOs with twister cables (Fig. 2)
to help control alignment until an appropriate age for surgical correction is reached, usually around 6 years [18].
AFOs with twister cables can be introduced as early as
2 years of age. In addition, in patients who are too young forosteotomies who develop excessive valgus stress at the knee
joint due to rotational malalignment, a knee–ankle–footorthosis (KAFO) with a free knee joint may be used [18].

HipHip deformity in patients with spina bifida results frommuscle imbalance and paralysis around the hip joint andmay present as contracture, subluxation, or dislocation [4].

Fig. 2 a Ankle–foot orthosis (AFOs) with twister cables, frontal
view. b AFO with twister cables, side view

If not treated properly, contractures can lead to pelvic
obliquity and compensatory spinal abnormality [4]. Paralytic hip dislocation is a common and complicated problem. In the past, transfer of the iliopsoas tendon along with
open reduction and capsular plication was used in patientswith spina bifida in order to achieve and maintain reduction

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of paralytic hip dislocations. However, current treatment
goals based on studies of functional results focus on
maintaining hip range of motion with contracture release
only [26].
Many of the earlier studies reporting on the results ofmajor hip reconstruction in spina bifida patients classified
success or failure solely on the basis of radiological results
demonstrating the maintenance of hip reduction. The
functional consequences of such an extensive surgicalprocedure for patients with spina bifida were often notassessed. Concerns developed regarding whether theradiological success of hip reduction led to restricted rangeof motion and pathologic fractures, thus, compromising thefunctional result [27].
Subsequent studies of functional results found that thepresence of a concentric reduction did not lead to improved
hip range of motion or the ability to ambulate. One seriesof 76 patients compared the functional results in those who
had undergone surgical treatment to reduce the hip to those
who had not [28]. The authors found that the presence of aconcentric reduction did not lead to improved hip range of
motion or ability to ambulate, nor did it provide a decreasein pain or need for bracing.
Another series examined 20 patients with low-lumbar
spina bifida who walked with crutches and underwent
three-dimensional gait analysis to determine the influenceof unilateral hip dislocation on gait [22]. The authors
reported that the walking speed of patients with dislocatedhips was 60% of normal, which corresponds to the walking
speed in low-lumbar patients without hip dislocation inprevious studies from the same center. The authors also
noted that gait symmetry corresponded to either theabsence of hip contractures or bilateral symmetrical contractures. They found no relationship between gait symmetry and hip dislocation and concluded that there is no
indication for surgical relocation of the unilaterally unstable hip. Rather, they recommend correcting unilateral soft
tissue contractures in order to restore gait symmetry.
Many authors have noted a high rate of complications
leading to decrease in ambulatory function in patients treated surgically for the reduction of hip dislocation. One series
reported a high rate of complications, including loss of
motion (29%) and pathologic fractures (17%), in a surgicallytreated group [28]. In another series, 36% of patients had
worsened ambulatory capacity as a result of surgical complications [29]. Another series reported that 11% of patients
had a worsening of their neurological deficit after surgery to
reduce the hip [27]. Although surgical treatment may allow
reduction of the dislocated hip, this result must be weighedin terms of the potential for complications and functional
decline. Another factor is the likely need for subsequent
procedures to maintain reduction and the effects of prolonged treatment on the patient and family [30].

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Multiple authors have concluded that the most importantfactor in determining the ability to walk is level of neural
involvement and not the status of the hip [22, 27–29, 31].
In a review comparing 30 patients with no surgical treatment of hip dislocation with 11 patients who had surgicaltreatment, the authors found that the ability to walk was
independent of hip reduction, but, instead, depended onneurological level [29]. The authors of the previously
reported comparative review came to the same conclusion[28]. Correspondingly, authors have stressed that thepreservation of muscle strength, specifically of the iliopsoas and quadriceps, is more relevant to determining
potential for adult ambulation than the status of the hip
joint. Treatment goals should include a level pelvis andfree motion of the hips rather than radiographic reductionof the hip [28]. Especially in high dislocations and older
children, the only recommended surgical treatment is
contracture release [22, 28].
An important consideration not addressed by any of thecurrently existing studies is the question of how to treat therare sacral-level patient with a dislocated hip who walks
without support. These patients may demonstrate anincreased lurch due to the loss of a fulcrum resulting fromthe dislocated hip. Consequently, these patients may benefit from surgical reduction, and further studies are necessary to examine this issue.

Knee
Patients with spina bifida may have involvement of theknee joint in the form of knee flexion or extension contracture, knee valgus deformity, or late knee instability andpain. Contractures occur most commonly in patients withlower thoracic and high lumbar lesions, and less often inpatients with lower lumbar lesions [4]. Deformity at theknee joint results from many factors, including static forcesof positioning, fibrosis of surrounding muscles, muscle
imbalance around the knee joint, and fracture malunion.
Knee flexion contractures may occur in both ambulatory
and non-ambulatory patients, but tend to be of greater
magnitude in thoracic-level as opposed to lumbar-level
patients. Many factors contribute to the development ofknee flexion contracture, including gradual contracture of
the hamstrings with contracture of the posterior knee capsule due to quadriceps weakness and prolonged sitting,spasticity of the hamstrings due to tethered cord, andquadriceps weakness with paralysis of the gastrocnemiussoleus and gluteus muscles [18]. In ambulatory patients,
flexion deformity of greater than 20° can interfere withorthotic fitting and ambulation [32]. Increased knee flexion
during gait causes increased oxygen cost and less efficientambulation [23]. Non-ambulatory patients can usually

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tolerate a greater degree of contracture without severe
limits on mobility and transfer. Flexion contractures thatinterfere with ambulation or with transfers or sitting balance in non-ambulatory patients respond well to radicalknee flexor release including the hamstrings, gastrocnemius, and posterior capsule. In a prospective review of 45
knees with flexion contracture in patients with spina bifida,
the mean knee flexion contracture decreased from 39°
before surgery to 5° after surgical release with an averagefollow-up of 13 years [33]. The authors noted improvement in gait after surgical release, especially in low-lumbar
level patients, and noted recurrence most commonly inthoracic-level patients.
Knee extension contracture is less common than knee
flexion contracture and may occur secondary to unopposed
quadriceps function with weak hamstrings, extensive
bracing in extension, or surgical treatment for flexion
contracture [2]. However, most cases are congenital andoccur bilaterally. Often, there are associated congenital
anomalies such as an ipsilateral teratologic hip dislocation
or clubfoot [18]. Initial treatment consists of serial casting
attempting to achieve at least 90° of knee flexion, which is
successful in most patients. For patients with persistentextension contracture that interferes with gait, a VY
quadriceps plasty has been shown to successfully improve
gait and sitting. One study reported that 13 of 15 patientstreated with VY quadriceps plasty maintained at least 90°
of flexion at 43 months follow-up [32]. In non-ambulatory
patients in whom extension contracture causes difficultywith sitting and transfers, tenotomy of the patellar tendon isan option. In a series of eight patients without active
quadriceps function, the authors noted 50–70° of knee
flexion with tenotomy of the patellar tendon and 90° or
more of flexion with division of the medial and lateralretinacula as well [34]. A successful result was achieved infive out of eight patients without further surgery required atfollow-up of at least 4 years. The authors stress that thistechnique is recommended only for patients without normal quadriceps function and they would, otherwise, would
perform a formal quadricepsplasty.
Another problem seen frequently in spina bifida, especially in low-lumbar and sacral-level patients, is valgus
knee deformity leading to instability, pain, and arthritis in
adulthood. A study of 72 community ambulators greaterthan 23 years of age found that 17 (24%) had significant
knee symptoms [35]. Gait analysis has allowed the identification of multiple factors contributing to abnormal
valgus stress, including rotational malalignment of thefemur, femoral anteversion in association with excessive
external tibial torsion, excessive trunk and pelvic movement, and knee flexion contractures [10, 24, 36, 37]. Surgical correction of excessive rotational deformities isindicated in patients over the age of 6 years and has been

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shown to lead to a significant improvement in knee stressand pain and may prevent the onset of late degenerativechanges [21]. If knee valgus is associated with knee flexion
contracture or hindfoot valgus, the surgical correction ofthese deformities is required at the same setting. In addition, the use of an AFO, forearm crutches, or a combinationof both should be encouraged to increase stance-phasestability and decrease stress in the knee joint [24, 36].

Rotational deformities
Both ambulatory and non-ambulatory patients with spina
bifida frequently develop torsional deformities of the lower
extremities involving the femur and/or the tibia. The
femoral torsion present at birth in all newborns does notdecrease normally with growth in a child with spina bifida
due to the presence of abnormal gait and activity levels [4].
Tibial torsion is even more common in patients with spina
bifida than femoral torsion. Tibial torsion can be either
external, which is acquired secondary to muscle imbalance,or internal, which is congenital and frequently associatedwith clubfoot deformity.
For non-ambulatory patients, torsional deformities arelargely a cosmetic problem. Initially, ambulatory patientscan be treated conservatively with orthoses such as AFOs
with twister cables. However, many ambulatory patients
will go on to develop severe femoral and tibial rotational
deformities, which can lead to labored gait, difficulty withorthotic fitting resulting in skin ulceration, and pain [21].
For ambulatory patients, the goal is to minimize bracing
requirements while achieving as normal a gait as possible[38]. A careful assessment of the patient’s gait, including
computerized gait analysis if available, should be done todetermine the extent of deformity correction needed before
recommending rotational osteotomy.
Internal tibial torsion causes gait disturbance when thefoot of one extremity catches on the contralateral side
during swing [2]. Internal torsion can be treated by rotational osteotomy using the technique described previously[39]. We have found successful healing using this technique with a few modifications. We perform the osteotomy
distally using a drill first to create multiple holes along thepath of the intended osteotomy and then complete theosteotomy with a saw in the hope of decreasing thermal
insult to the bone. In addition, we fix the osteotomy using a
dynamic compression plate to provide stable fixation.
External rotation deformity may result from the hip but
is more commonly due to external tibial torsion, whichinterferes with gait and causes difficulty with orthotic fit.
The external rotation of the tibia places the medial malleolus in the line of progression and can cause rubbing
against the AFO, leading to skin breakdown [2]. Treatment

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such as skin breakdown and post-immobilization fractures.
Unless absolutely necessary, the use of a spica total body
cast should be avoided. A custom-molded total body splint
may be used as an alternative to a spica cast, even inpatients who have undergone bony surgical procedures(Fig. 3). Rigid internal fixation should be used rather than
Kirschner wire fixation in order to decrease the risk of nonunion, allow early mobilization, and allow early weightbearing. Post-operative physical therapy should begin assoon as wounds are stable and signs of healing are present
in order to work on active and passive range of motion andinitiate early weight-bearing. Once immobilization is discontinued, crawling should be strictly forbidden for at least
3–4 weeks to prevent the risk of fracture.

Conclusion

Fig. 3 Custom-molded total body splintof this deformity with internal rotational osteotomy of thetibia should be considered when external torsion is greaterthan 20° [40]. We use the same technique as for correcting
internal torsion. In addition, it is essential to examine thepatient’s entire lower extremity, paying particular attention
to the hindfoot, as we have noticed a correlation between
external tibial torsion and hindfoot valgus. Often, bothdeformities require treatment in order to achieve a successful result. Treatment of the hindfoot valgus consists ofa medial sliding osteotomy of the os calcis.
Studies have reported successful results in terms of gaitparameters and range of motion in 80–90% of patientstreated with lower extremity osteotomies [38, 41]. Additionally, derotation osteotomy may delay or prevent theonset of late degenerative changes about the knee forpatients with excessive external tibial torsion [21]. One
study found increased valgus knee stress in eight out ofeight patients with external tibial torsion [21]. A significant
improvement in the abnormal knee moment was seen after
derotational osteotomy. However, serious considerationmust be given to the increased risk of complications inpatients with spina bifida undergoing tibial osteotomies,
including delayed union and wound infection [41].

Post-operative care
For patients with spina bifida, care must be taken during
the post-operative period to prevent known complications,123

The orthopedic care of patients with spina bifida is bothchallenging and rewarding. Due to the many medicalcomorbidities involved, careful evaluation and management of these patients should occur as part of a teamapproach involving members from multiple specialties,
including urology, neurosurgery, pediatrics, physiatry,orthotics, physical therapy, and social work. As part of thisteam, the goal of the orthopedic surgeon should be tominimize deformity and maximize function and mobility,
while limiting complications.

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