SPINAL DYSRAPHISM
20
REHABILITATION OF CHILDREN
WITH SPINAL DYSRAPHISM
The purpose of this article is to discuss
advances in the comprehensive rehabilitation
management of children with spinal dysraph-
ism. The complexity of these children necessi-
tates that this management be provided by a
team consisting of multiple medical, surgical,
and allied health professionals. Specific issues
pertinent to the ongoing comprehensive man-
agement of children with spinal dysraphism
include (1) prevention of complications; (2)
mobility; (3) toilet training and management
of the neurogenic bladder and bowel; (4) skin
management; (5) health care maintenance; (6)
assessment of cognitive, language, and fine
motor skills; (7) provision of appropriate ther-
apy to enhance gross motor, fine motor, and
communication skills; (8) school and voca-
tional integration; (9) psychosocial adjustment
to disability; (10) family adjustment; (11) rec-
reation and avocational activities; and (12)
transition from adolescence to adulthood.
REHABILITATION GOALS AND
TREATMENT STRATEGIES
The general rehabilitation goals for chil-
dren with spinal dysraphism include im-
provement of function and independence,
normalization of the child's developmental ex-
perience to the extent possible, improvement
From the Departments of Physical Medicine and Rehabilita(tion and Pediatrics, University of California, Davis Medical
Center, Sacramento, California
NEUROSURGERY CLINICS OF NORTH AMERICA
VOLUME 6 NUMBER 2 . APRIL 1995
1042-3680/95 $0.00+ .
Craig M. McDonald, M.D.
of quality of life and self-esteem, ongoing
education of affected children and caregivers,
and provision of anticipatory guidance. Reha-
bilitation treatment strategies to lessen or
eradicate disability include (1) prevention or
correction of additional impairment or dis-
ability; (2) enhancement of systems unaf-
fected by the pathologic process; (3) enhance-
ment of the functional capacity of affected
systems; (4) use of adaptive equipment and
aids to promote function; (5) modification of
the social, educational, and vocational envi-
ronment; and (6) psychological techniques to
enhance patient performance and adjust-
ment.lo2An ideal comprehensive rehabilita-
tion program should include sufficient train-
ing, education, and long-term monitoring to
enable the child not only to obtain self-suffi-
ciency, but also to maintain an optimal level
of function throughout life.
CASE FOR INTERDISCIPLINARY
MANAGEMENT
A coordinated, interdisciplinary team ap-
proach is the most effective way to provide
care and ensure the achievement and mainte-
nance of optimal function for children with
spinal dysraphism. Team members within a
myelodysplasia clinic usually include spe-
393
394 McDONALD
cialists in pediatrics or pediatric physical
medicine and rehabilitation, pediatric neuro-
surgery, pediatric urology or neuro-urology,
pediatric orthopedics, pediatric nursing,
physical and occupational therapy, and medi-
cal social work. One physician usually as-
sumes the role of clinic coordinator. The inter-
disciplinary approach is characterized by
effective communication between various
team members with regard to their evaluation
and management plan. For example, it may
be detrusor hyperreflexia identified on urody-
namics by the urologist, spasticity of the pos-
terior tibialis recognized by the orthopedist,
or interval change in strength noted on man-
ual muscle testing by the physical therapist
that provides the pediatric neurosurgeon
with the initial clinical evidence of progres-
sive ascending myelopathy. The array of pro-
fessionals and specialty teams involved in the
complex life course of a child with myelodys-
plasia is not limited to medical disciplines
and have been described in detail by Shurt-
leff.95
Kaufman and colleagues51 have reported
the negative effects of disbanding a multidis-
ciplinary myelomeningocele clinic. Despite
the continued availability of the same spe-
cialty care in the same institution, approxi-
mately one third to one half of myelomenin-
gocele patients from a disbanded clinic had
no identified caregiver in each of the principal
specialties, and 45% to 66% of patients failed
to have regular medical and specialty care.
This lack of care in patients with the least
follow-up was associated with an increase in
serious morbidity, including such complica-
tions as nephrectomy, amputation, and severe
decubitus ulcers. The complication rate was
found to be lower in control patients followed
in a multidisciplinary myelodysplasia clinic.
A common problem following closure of the
multidisciplinary myelomeningocele clinic
was failure of primary physicians to assume
the responsibility of coordination of patients'
comprehensive care.
PREVENTION OF COMPLICATIONS
Prevention of complications is necessary
for the child with spinal dysraphism to real-
ize his or her maximum functional potential.
Other articles in this issue are devoted to
prevention of neurologic, renal, and orthope-
dic complications. Rehabilitation profession-
als and therapists who evaluate the child seri-
ally can provide objective evidence for
worsening spasticity, functional decline, de-
creased strength or fine motor skills, and new
sensory deficits, which might be early indica-
tors of progressive myelopathy or brain stem
dysfunction. Quantitative strength measures
can be applied to children as young as age 6
and appear to be more sensitive for demon-
strating strength loss than manual muscle
testing when strength is grade 4 to 5. In gen-
eral, by the time strength declines to grade 4
by manual muscle testing, isometrically mea-
sured strength is only 40% to 50% of normal
control values.2, The reliability of isometric
measurements in children with myelomenin-
gocele appears to be adequate for most mus-
cle Grip strength measurements by
hand-held dynamometry also provide useful
objective information in myelomeningocele.5s
The Jebson Taylor Hand Function Test1OSis
a standardized measure of fine motor skill
applicable to myelomeningocele children for
serial objective measurement.
MOBILITY IN SPINAL DYSRAPHISM
Mobility Prognosis
One of the first questions that parents ask
when discussing their newborn with myelo-
dysplasia is "will he or she eventually walk?"
The clinician may give fairly accurate prog-
nostic information regarding future mobility
depending on the pattern of paralysis. It is
perhaps more useful to help the family de-
velop an emphasis on functional and devel-
opmentally appropriate mobility that may oc-
cur using a variety of methods as opposed to
establishment of upright standing and ambu-
lation as the highest priority.
Multiple factors have been proposed to
contribute to mobility status in myelodys-
plasia:
Neurologic motor level
Sensory level/proprioceptive deficits
Hip flexion contractures
Knee flexion contractures
Hip subluxation/dislocation
Foot/ankle deformity
Spinal deformity
Fractures
Spasticity
Degenerative joint complications
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM
Upper extremity dysfunction
Poor balance/ataxia
Use of effective braces/assistive devices
Obesity
Age
Intelligence
Patient motivation
Family motivation
Medical staff philosophy
Investigators have repeatedly found that
level of neurologic lesion and associated
strength of lower extremity muscles are the
most important factors influencing whether
children with myelomeningocele attain func-
tional ambulation,4,6, 7, 19, 27. 34, 43, 44, 69, 87, 101, 103
The use of specific neurosegmental motor le-
sion level is not ideal for prediction of ambu-
latory outcome for several reasons. McDonald
and associates71have demonstrated that clini-
cally determined patterns of muscle strength
in children with myelodysplasia often vary
from the classic neurosegmental level de-
scribed by Sharrard.91For example, the me-
dial hamstrings were frequently found to be
stronger than the anterior tibialis and were
noted to be present in some patients with
absent function of the anterior tibialis. Grade
4-5 iliopsoas is associated with strong quadri-
ceps and rarely observed with weak quadri-
ceps. The glutei appear to have significant L-
4 contribution given the finding that they are
frequently weakly present in association with
weak ankle dorsiflexion. There is also consid-
erable variability in ambulatory status of pa-
tients within particular neurosegmental le-
sion le~els,2~,
69 with most variability occurring
in the midlumbar segments.R7Samuelsson
and Skooga7believe that inconsistent classifi-
Figure 1. Percentages of patients (n= 87)
with iliopsoas strength 5 grade 3 who are
nonambulators, partial (household) ambula-
tors, and community ambulators. (From Mc-
Donald CM, Jaffe KM, Mosca VS, Shurtleff
DB: Ambulatory outcome of children with my-
elomeningocele: Effect of lower-extremity
muscle strength. Dev Med Child Neurol
33:482-490, 1991; with permission.)
395
cation criteria for neurosegmental level and
the use of broad categories account for the
large disparity in ambulatory status reported
in patients with similar neurosegmental le-
sion levels.
Previous authors have stressed the impor-
tance for ambulation of strong iliops0as,6~
quadriceps,4, 34, 44, 69, 89, 103 gluteuS medius,44, 69
and ham~trinns.~ In a studv of 291 children
with myelom&ingocele, ~ c ~ o n a and l d co-
workers" found that the specific strengths of
iliopsoas, quadriceps, gluteus maximus and
medius, and anterior tibialis explained 86O/0
of the observed variance in mobility outcome.
Other proposed determinants to ambulation
contribute little independently to the walking
status of children with myelomeningocele.
For example, presence of hip dislocation is
not an important independent determinant of
mobility status." Other factors documented
to influence ambulatory status include obe-
sity for the thoracic and upper lumbar
groups4; spasticity or poor balance related to
the myelodysplasia, syringohydromyelia,
Chiari malformation, or other central nervous
system malformation", lo" a combination of
hip flexion deformity, pelvic obliquity, and
s ~ o l i o s i s ~and
~ ~ ;age.4,19* 87, lol Ambulatory
"9.
outcomes by specific muscle strength based on
serial assessment for patients with myelomen-
ingocele are shown in Figures 1 through 3.
Thus, the use of specific patterns of lower
extremity strength is recommended for pre-
dicting walking ability and establishing am-
bulatory goals. Prognostication of mobility
for the infant, however, is never certain. The
stability and reproducibility of manual mus-
cle testing measurements increase from birth
" N o n Ambuta!ors P ,I a' Community
Ambiil?!orn Ambutalore
[--3Itlopsoas 0-1 F( Ifiopsoas 2-3
396 McDONALD

Figure 2. Percentages of patients (n = 159)

with grade 4 to 5 iliopsoas and grade 4 to
5 quadriceps who are nonambulators, partial
*
0
40
(household) ambulators, and community am-
30 bulators. Proportions with grade 0 to 2 versus
@ 20 3 to 5 glutei and anterior tibialis are also
shown. (From McDonald CM, Jaffe KM,
10
0 Mosca VS, Shurtleff DB: Ambulatory outcome
0 of children with myelomeningocele: Effect of
Mon Ambulstota Partgat Comrnunlty
Ambulators Ambutatare lower-extremity muscle strength. Dev Med
QluteilAnt Tib QluteilAnt Tib Child Neurol 33:482-490, 1991; with permis-
Qrede 0-2 amdo 3-6 sion.)

to age 5 or 6 when peak stability is reached.70 mented that both positive and negative mo-
Some muscle groups such as the gluteus me- bility changes occur from age 5 years to ado-
dius and gluteus maximus are particularly lescence. Changes in mobility were most
difficult to evaluate reliably in the newborn. common in those with strong " ilio~soasand
I

Although anterior tibialis function, if present quadriceps and weak or absent gluteus me-
in the newborn, correlates with presence of dius and gluteus maximus. Stillwell and Me-
some gluteal strength, absent anterior tibialis nelauslol found that adults with s ~ i n abifida
I

function does not exclude the possibility of tend to be either community ambulators or
some gluteus medius function being pres- nonambulators. Those who achieve house-
ent.71 hold ambulation do not usually continue to
ambulate for short distances as adults.
Mobility status is ultimately determined by
Age Effects and Energy Expenditure the energy expenditure required to ambulate.
for Mobility Those with ~arazlaresis
I I
ultimatelv select a
J

comfortable walking speed to minimize the

Asher and Olson4 found that changes in
ambulatory status over time were usually
negative and were related to lack of motiva-
tion, obesity, and musculoskeletal deformity.
Most studies have failed to show a common
age for deterioration in mobility status.4,6y,
87,101 In fact, McDonald and coworkers6y
docu-
rate of energy consumption. To maintain this
comfortable level of energy
-- expenditure dur-
ing ambulation, they move more slowly.
Wheelchairs allow individuals to travel at
speeds comparable to that of able-bodied
walking with equivalent energy expenditure.
Agre and associates1 found walking in chil-

USE AIDS/BRACES aNO AIDS/BRACES

Figure 3. Percentages of community ambula-

tors who rely on aids or braces as function of
gluteus medius strength. (From McDonald
CM, Jaffe KM, Mosca VS, Shurtleff DB: Am-
bulatory outcome of children with myelomen-
ingocele: Effect of lower-extremity muscle
0-1 2-3 4-5 strength. Dev Med Child Neurol 33:482490,
GLUTEUS MEDIUS STRENGTH 1991; with permission.)
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM
dren with myelomeningocele to require ap-
proximately twice the energy expenditure
(expressed in amount of 0, consumed per
minute) compared with propelling a wheel-
chair at an equivalent speed. In a study of
myelodysplasia patients, Flandry and col-
l e a g u e ~showed
~~ an advantage to reciprocal
gait with the reciprocating gait orthosis
(RGO) versus swing-through gait both in
terms of net energy consumption rate (ex-
pressed in amount of 0, consumed per mi-
nute) and energy cost (amount of 0, con-
sumed per meter traveled). Others also report
advantages to the RGO in terms of improved
ambulatory velocity, energy expenditure, and
proportions of patients achieving community
ambulatory status.67,'I4 Care must be taken
to evaluate actual ambulatory function. One
study2$ focusing on actual ambulatory dis-
tances in thoracic and high lumbar myelo-
meningocele patients found that 94% never
walked as far as one block. In those classified
as community ambulators, 56% walked around
a block without stopping at a frequency of
less than once a week.
Controversy of Walking Versus
Wheelchair Use in Myelomeningocele
Controversy exists as to whether high-level
myelomeningocele children should be pre-
scribed wheelchairs at a young age or encour-
aged to ambulate with parapodium or swivel
walker and subsequently a hip-knee-ankle-
foot orthosis (HKAFO) or RGO. One ap-
proach is geared toward the aggressive
achievement of upright ambulation and pre-
sumes psychological and physiologic benefits
to upright posture. The other approach offers
a variety of aids and devices (including
wheelchairs) but does not aggressively strive
for ambulation. Only two studiess9h3 have
been specifically designed to compare specific
outcomes in a program oriented to walking
with similar outcomes observed in matched
children using wheelchairs from an early age.
The proposed benefits of upright ambulation
are described along with brief reviews of rele-
vant literature:
1. Increased bone densityldecreased fractures.
Literature suggests bone density in my-
elomeningocele to be directly associ-
ated with neurosegmental innervation
and volitional muscle power. When
397
controlling for lesion level, there ap-
pears to be no difference in bone den-
sity by single-photon absorptiometry
between those ambulating and those
using only a ~ h e e l c h a i r .However,
~~
Mazur and colleagues6%howed that
~atientswho ambulated had fewer
iractures than those prescribed a
wheelchair early in life. Proponents of
wheelchairs argue a contrary point; i.e.,
children who use wheelchairs are
subjected to fewer surgical procedures
with cast immobilization-a known
risk factor for fractures. L iI~ t a kand col-
leagues59showed no difference in frac-
tures between two programs empha-
sizing early ambulation and early
wheelchair use.
2. Contractures. No difference in hip
flexion contractures was observed be-
tween early ambulation and early
wheelchair use by Liptak and col-
l e a g u e ~Wright
. ~ ~ and coworker~l'~ and
Liptak and colleagues" demonstrated
that those using wheelchairs early in
life had greater
- knee flexion con-
tractures.
3. Urinary tract. Upright posture and
walking have been theorized to pro-
mote better drainage of the urinary
tract, reducing complications such as
urinary tract infections and hydro-
nephrosis. Liptak and colleaguess9
showed no advantage in this regard to
early ambulation, and no studies to
date show significant differences in uri-
nary tract outcomes between upright
ambulators and wheelchair users when
controlling for lesion level.
4. Bowel management. Liptak and col-
l e a g u e ~ showed
~~ that children in
wheelchairs had fewer fecal accidents
than children using the upright para-
I
odium. This was attributed to both
mechanical compression of the anus
during sitting and increased abdominal
pressure of those standing in a brace.
5. Cardiovascularfitness. No studies to date
have addressed this proposed benefit
to ambulation in myelomeningocele.
6. Obesity. Contrary to expectations, Lip-
tak and colleagues59found parapodium
users in Rochester to be more obese
than wheelchair users in Seattle. This
was attributed to the presence of a nu-
tritionist in the Seattle Clinic and
398 McDONALD
greater time spent watching television
by the Rochester children.
7. Decubitus ulcers. No differences in fre-
quency of skin breakdown have been
observed in those standing versus
wheelchair users.59Children in a stand-
ing program were more likely to de-
velop skin breakdown of the foot and
distal lower extremity, whereas chil-
dren using wheelchairs were more
likelv to develo~skin breakdown of the
sacrum, ischiurn, and perineum.
8. Activities of daily living. Selected mea-
sures of independence, including hy-
giene, dressing, transfers, and bowel
and bladder independence, were not
shown by Liptak and colleagues59to be
different for ambulators versus wheel-
chair users. Mazur and coworkers'j3
demonstrated no difference in perfor-
mance of basic activities of daily living
but improved transfer skills in those
patients who ambulated early in life.
9. Fine motor skills. No difference has been
demonstrated on standardized mea-
sures of hand function between those
provided a wheelchair versus matched
patients participating in a standing
program.'j3Franks,3O however, has dem-
onstrated a significant decline in visual-
motor performance immediately fol-
lowing 'ambulation in upper lumbar
and midlumbar myelomeningocele pa-
tients. This decline has been attributed
to performance decrements observed in
those experiencing fatigue from inter-
mittent near-maximal aerobic activity."
10. Socialization. There does not appear to
be a significant advantage to early pro-
vision of a wheelchair or standing pro-
gram with regards to sports or extra-
curricular activity parti~ipation.~~ Other
issues such as peer relations and social
isolation have not been studied with
adequate design.
11. Utilization o f health seuvices. Mazur and
associates" found that patients who al-
ways used a wheelchair spent fewer
days in the hospital than those who
had participated in a walking program.
Ambulatory children were admitted
more freauentlv
I
for osseous and soft
tissue procedures to correct musculo-
skeletal alignment problems. Liptak
and c0lleagues,5~ however, found no in-
crease in days of hospitalization in am-
bulatory children.
Developmental Theory and Self-
Produced Locomotion
Developmental research suggests that self-
produced locomotion is the primary vehicle
in the young child for exploration, learning,
socialization, and development of indepen-
dence and feeling of self-competency." Stud-
ies indicate that mobility restriction can result
in a persistent pattern of passive, dependent
behavior-specifically a lack of curiosity and
initiative.ll Telsrow and associates106fomd
that infants with myelomeningocele showed
delayed acquisition of fundamental cognitive
skills until the point at which crawling was
achieved. Landry and associates54 found
spina bifida children spent less time using
goal-directed behaviors and more time in
simple manipulation of toys compared with
normal children. An association was found
between goal-directed behaviors and per-
ceived self-competence in spina bifida chil-
dren. Butlerlofound a change in behavior con-
sistent with increased self-initiative in young
children with physical impairments (aged 18
to 39 months) when they experienced inde-
pendent locomotion with powered wheel-
chairs.
Mobility in high-level myelomeningocele
should not be viewed as a strict choice be-
tween standing or wheelchair. A functional
mobility program may concurrently provide
physical therapy, appropriate orthotic de-
vices, orthopedic surgery for alignment prob-
lems, special mobility devices, ambulatory as-
sistive devices, and a wheelchair to meet the
different mobility needs of the child. Almost
all community ambulators with myelomenin-
gocele achieve unsupported sitting by 15
months, crawl on all fours before age 18
months, pull to stand by 24 months, and am-
bulate outside by age 4.28,100 For the 2-year-
old child with low probability for community
ambulation, it is most appropriate to provide
a wheelchair and not delay this prescription
until school entry. When multiple options are
available, children do not give up ambulation
entirely but rather select the most appropriate
means of mobility for the particular activity
at hand, taking into account energy require-
ments, time, social situation, and the people
with whom they are involved.ll
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM 399

MOBILITY MANAGEMENT BY
PATTERN OF PARALYSIS

Thoracic and High Lumbar Level

(Iliopsoas and Quadriceps Grade 0-3)71

Little controversy exists regarding the ulti-

mate mobility status in children with thoracic
and high lumbar level paralysis. One hun-
dred percent of these children ultimately re-
auire-a wheelchair for all or part of their
1
mobility. Reported percentages of community
ambulators range from O0/Oz0, 43, 69, 86 to
33°/0.h3,lol If head and trunk control are de-
layed, early therapy is geared toward devel-
opment of postural mechanisms of support.
It may be useful to substitute passive trunk
s u ~ ~ otor facilitate
I I
t development of bimanual
manipulatory skills, dexterous hand use, and
hand-eye coordination. These children may
be fitted with a Skinner sitting orthosis (Fig.
4) or hip positioning orthosis brace5 at the
time they are developmentally ready to
achieve sitting balance. The caster cart57has
been found to be useful for the paraplegic
child under 18 months old for development
of upper extremity strength and control of
trunk and head. Once the child has developed Figure 5. Swivel walker used in early standing program
during the pre-school years (Courtesy of Wallace M. Mot-
good head control and sitting balance, a lock, CO, BS, Center for Orthotics Design, Redwood
City, CA.)

standing program during the preschool years

Figure 4. Skinner sitting orthosis to provide passive trunk
support and encourage development of upper extremity
skills. (Courtesy of Wallace M. Motlock, CO, BS, Center
for Orthotics Design, Redwood City, CA.)
is advocated at many centers. Typically a
standing frame, p a r a p o d i ~ r n , ~ or~ swivel
walker7"Fig. 5) is prescribed. Forward loco-
motion in a parapodium or swivel walker can
be accomplished in younger children with
adequate balance. Physical therapy is re-
quired for gait training. Older children with
good trunk control and upper body strength
can ambulate short distances with a swing-
through gait pattern and HKAFOS'~,~~ or with
a reciprocal gait pattern using a RG0.64,67
The RGO was originated by Motlock, and
further refined by Christian, Fillauer, Doug-
las, and M o t l o ~ k .75~ ~
It , dynamically couples
flexion of one hip with extension of the con-
tralateral hip and effectively compensates for
lack of hip extension power and the resulting
forward pelvic tilt and lordotic posture. Crite-
ria for successful ambulation with the RGO
have been proposed by Guidera and associ-
a t e ~These
: ~ ~ criteria include:
400 McDONALD
No obesity
Minimal spinal deformity
Absence of spasticity
Good upper extremity strength
Good trunk and standing balance
Knee contractures < 30 degrees
Hip contractures < 45 degrees
Motivated family and patient
Lumbar level lesion
Symmetric motor function
Symmetric hip position (dislocation)
Previous standing/walking
No mental retardation
Able to travel to orthotist for repairs
The author prefers the Isocentric RGO
brace (Center for Orthotics Design, Redwood
City, CA) developed by Motlock (Fig. 6A,B).
Hip abduction joints may be added to this
orthosis to facilitate intermittent catheteriza-
tion (Fig. 6C).
In the author's experience the RGO does
not work well in children with hip flexion
contractures greater than or equal to approxi-
mately 40 degrees. In addition, knee flexion
contractures greater than 25 to 30 degrees
are quite difficult to accommodate because
of excessive forces transmitted to the patella.
Successful use of the RGO strongly depends
on the presence of useful hip flexion power,
and several authors urge restraint with re-
gard to iliopsoas soft tissue releases to correct
hip flexion deformity.67,"4
Similarly, extreme caution should be used
with regard to orthopedic soft tissue releases
in older thoracic level patients to accomplish
a goal of upright ambulation. These con-
tractures are not likely due to muscle imbal-
ance or spasticity but rather prolonged static
positioning in flexion. In patients who have
been using a wheelchair for a prolonged pe-
riod, fixed knee flexion contractures may be
due to nonambulation and have not necessar-
ily caused the child to cease walking.l13 These
patients may have a low likelihood of achiev-
ing functional ambulation after release of con-
tractures.
As the thoracic level child without useful
hip flexion approaches the age of 24 to 36
months, it is appropriate to consider prescrip-
tion of a lightweight manual wheelchair to
be used concomitantly with standing devices.
The physical therapist can assist with transfer
skills from floor to wheelchair. Rarely the
more severely impaired child with significant
complications of hydrocephalus or central
nervous system malformation demonstrates
significant upper extremity involvement and
balance disturbance and requires a power
wheelchair for independent mobility. Chil-
dren as young as 24 months developmental
age can learn to drive a motorized wheel-
chair safely.12
Midlumbar Level (Iliopsoas and
Quadriceps Grade 4-5; Medial
Hamstring Grade 0-4; Anterior
Tibialis and Glutei Grade 0-2;
Gastrocnemius and Soleus Grade 0)"
A high percentage of these children achieve
community ambulation with adequate brac-
ing. Few ambulate without bracing. Early in
life, these patients often have dynamic inter-
nal tibial torsion secondary to imbalance be-
tween the medial and lateral hamstrings. In
younger patients, this torsion can usually be
partly and temporarily corrected by provision
of twister cables that externally rotate the
limb at the hip joint. Fraser and Menelaus31
recommend closed osteoclasis of the tibia if
walking is impeded by excessive internal tib-
ial torsion at a young age. If excessive rota-
tion and instability occurs at the hip (particu-
larlv with dislocation) HKFOs or a RGO
-I
provides more optimal alignment.
In older patients, fixed external tibial tor-
sion is more common in combination with
~ -
genu valgum, ankle hindfoot valgus, and pla-
nus of the medial longitudinal arch. Children
exhibit crouched gait caused by absence of
srastrocnemius-soleus function, which nor-
"
mally prevents anterior translation of the tibia
relative to the foot during midstance. This
excessive anterior tibial translation can be
partly alleviated by a floor reaction ankle foot
orthosis (Fig. 7), which decreases crouch gait
by preventing ankle dorsiflexion and main-
taining a knee extension moment during
stance. These children all ambulate with a
waddling gait or gluteus medius lurch and
thus benefit from a wheeled walker or loft-
strand crutches. Occasionally a knee-ankle-
foot orthosis (KAFO) with free knee is
provided to protect the medial collateral liga-
ment of the knee or provide proprioceptive
feedback to the child lacking L-4 sensation.
Risk of secondary trophic ulceration of the
medial foot with planovalgus foot deformity
necessitates orthopedic surgical management.
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM 401

Figure 6. lsocentric Reciprocating Gait Orthosis viewed posteriorly (A) and from the lateral position (B), which
dynamically couples flexion of one hip with extension of the contralateral hip. (C) Hip abduction joints in the lsocentric
Reciprocating Gait Orthosis that facilitate intermittent catheterization. (Courtesy of Wallace M. Motlock, CO, BS, Center
for Orthotics Design, Inc., Redwood City, CA.)
402 McDONALD
Figure 7. Floor reaction ankle foot orthosis to prevent
crouch gait in an L4 myelomeningocele patient.
Lower Lumbor-Sacral Level
(Iliopsoas, Quadriceps, and Medial
Hamstrings Grade 4-5; Anterior
Tibialis and Glutei Grade 3-5;
Gastrocnemius Grade 0-3)71
In those with strong (grade 4-5) iliopsoas
and quadriceps, the achievement of commu-
nity ambulation without occasional reliance
on a wheelchair is strongly associated with
antigravity gluteal strength. Knowledge of
gluteus medius, gluteus maximus, and associ-
ated anterior tibialis strength also helps in
the prediction of ambulation with or without
crutches and ankle foot orthotics (see Fig. 3).6y
More subtle crouch gait may be present sec-
ondary to ankle plantarflexion weakness and
responds to standard ankle-foot orthotics
(AFOs) or floor reaction AFOs. Provision of
KAFOs or a RGO is rarely necessary. Ortho-
pedic surgery is often required for achieve-
ment of a well-aligned plantigrade foot that
provides a stable base of support and mini-
mizes foot ulcerations.
Sacral Level (Partial Weakness of
Gluteus Maximus, Gastrocnemius-
Soleus Complex, and Foot Intrinsics)
All children are community ambulators.
Cavus and intrinsic foot deformities are com-
mon, and orthotics are rarely indicated.
TOILET TRAINING
Bowel and bladder incontinence is a major
social impairment for nearly all patients with
spinal dysraphism. Adults with myelomenin-
gocele have described inadequate toileting as
their most worrisome handicap.y6With ap-
propriate education and early family training,
application of appropriate urinary and bowel
programs based on individual pathophysiol-
ogy, and adequate patient/family compli-
ance, the proportion achieving social conti-
nence of both bowel and bladder varies from
8O0l0 to 100% depending on the severity of
myel~dysplasia.~~ The percentage of myelo-
meningocele patients achieving complete inde-
pendence in both urine and stool toilet training
is highly dependent on severity of neurologic
impairment; presence of physical deformity
that prevents the child from seeing and reach-
ing the perineum or transferring from wheel-
chair to toilet; presence of family disruption;
or a combination of failed compliance on the
part of family, school, or child. Approxi-
mately 60% of thoracic and upper lumbar
patients may require some ongoing assistance
for achievement of socially acceptable toilet
trainingy5 Partial or complete dependence
with toileting in myelomeningocele is
strongly associated with noncompetitive
work or unemployment.lo7
Initiation of clean intermittent catheteriza-
tion may be performed by the parent begin-
ning in the newborn period (if an excessive
leak point pressure is present on urodynam-
ics) or is initiated by the family during the
preschool years for attainment of social conti-
nence before the onset of school. Training the
family in a bowel program is ideally initiated
between ages 2 and 5 years. Before initiating
bowel training, the goal is to maintain a soft-
formed stool to prevent extensive anal stretch
and avoid complications of obstipation with
megacolon. Training of the child for indepen-
dent toileting skills begins between 4 and 10
years of age depending on severity of impair-
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM
ment and may incorporate use of pictures,
models, and anatomically correct dolls.
Neurogenic Bowel
Spinal dysraphic lesions proximal to or in-
volving S-2 and distal segments often lead to
absent sensation of rectal fullness, decreased
or increased baseline anorectal sphincter tone,
lack of volitional anorectal sphincter control,
and dyssynergia with intestinal peristalsis.
Defecation pathology can lead to constipation
(accumulation of stool), obstipation with
megacolon, and impaction. Some patients
with weak or nonexistent anal sphincters
from lower motor neuron paralysis may have
frequent passage of small amounts of stool
throughout the day. Diarrhea makes achieve-
ment of social continence in this setting par-
ticularly problematic. Goals are to prevent
potential complications and achieve social
continence using dietary control to achieve a
firm, high-bulk stool; evacuation of bowel at
a consistent time; and behavioral techniques
to improve compliance. Defecation patho-
physiology differs among patients with spinal
dysraphism, and hence one method of evacu-
ation cannot be optimally applied to all.
Methods include (1)digital stimulation with
or without manual removal of stool; (2) sup-
positories (e.g., bisacodyl) to stimulate peri-
stalsis; (3) irritative enema; (4) expansion en-
ema, often using a continence enema
(5) b i o f e e d b a ~ k " , ~ ~for
, ~ ~those
,"~
with partially intact sensation; and (6)
judicious and intermittent use of oral cathar-
tics used in combination with the above-men-
tioned techniques. Presence of the bulbocav-
ernosus and anal cutaneous reflexes
correlates significantly with achievement of
continence using standard techniques." The
enema continence catheter is an appropriate
option for those with decreased anal tone,
absent sacral cutaneous reflexes, and pre-
sumed lower motor neuron impairment.s8,
Neurogenic Bladder
The management of the neurogenic bladder
is discussed extensively in another article in
this issue. Planning for definitive surgical in-
terventions needs to take into account the
child's demonstrated independent self-care
403
behaviors and maturity because the patient
must maintain a proper schedule of bladder
emptying subsequent to surgical reconstruc-
tion.
SKIN MANAGEMENT
Paraplegia, anesthetic skin, musculoskeletal
deformity, soiling, and orthotic devices pre-
dispose patients with spinal dysraphism to
skin breakdown. Reported incidence per year
ranges from 25'/0~~ to 770/0.17 The costs associ-
ated with skin breakdown have been docu-
mented by Harris and The incidence
and location of pressure ulceration are di-
rectly related to functional motor 78
Patients with thoracic level paralysis and ky-
photic spinal deformity are at high risk for
skin breakdown over the dorsal spine. Tho-
racic and upper lumbar motor level patients
tend to develop sores involving the sacrum,
ischium, and perineum. These patients ac-
count for greater than 70% of accumulated
hospital time and overall cost expenditure^.^^
Okamoto and colleagues7R documented
higher rates of skin breakdown in association
with mental retardation, large head size, ky-
phoscoliosis, and chronic soiling. Low lumbar
and sacral myelomeningocele patients are
more likely to develop foot ulcerations. Foot
rigidity, nonplantigrade position, and perfor-
mance of surgical arthrodesis are strongly as-
sociated with eventual development of neuro-
pathic skin changes in the feet.62
It is critical to alert parents early on about
the need for careful skin monitoring and early
childhood training in basic fundamentals of
skin hygiene. Parents and children need to be
aware of the precise location of anesthetic
skin regions. Self-inspection techniques using
a mirror must be taught to children at an
early age. Children are taught to elevate their
bodies from their chairs regularly to enhance
capillary tissue perfusion through pressure-
sensitive tissues. Ill-fitting shoes or orthoses
must be immediately adjusted. Casts require
generous padding. These children are more
prone to burns and frostbite injury with expo-
sure. Pelvic obliquity must be carefully ac-
commodated by custom adaptive seating. Use
of a time-set alarm system as a pressure-relief
training device is quite helpful, and social
support in encouraging pressure relief should
come from family, peers, and the school sys-
tem. Advances in nonoperative wound heal-
404 McDONALD
ing include use of pulsed low-intensity direct
electrical current112and topical application of
recombinant platelet-derived growth fac-
tor~.~~
HEALTH CARE MAINTENANCE
Routine pediatric health care maintenance
is optimally provided by the local primary
care physician with consultation and educa-
tion available from members of the spinal
dysraphism multidisciplinary team. There
are, however, health care maintenance issues
specific to children with myelodysplasia that
deserve mention.
Feeding and Swallowing
During mfancy, it is critical to monitor the
child's oral-motor skills and feeding because
dysphagia may be a manifestation of hind-
brand dysfunction secondary to Chiari mal-
formation.81,s2 Although most with symptom-
atic brain stem dysfunction present in
the gradual onset of neurogenic
dysphagia in older children with Chiari I or
I1 malformation has been reported.81
Hearing and Vision
Strabismus has been noted in a significant
proportion of hydrocephalic spina bifida
children.45,lo9Radke and Goskys3reported the
incidence of conductive or sensorineural
hearing impairment to be 13% in their series
of myelodysplasia patients.
Developmental Assessment
Developmental assessment and provision
of appropriate anticipatory guidance is a criti-
cal component of primary care. With regard
to self-care skills and motor milestones in
myelomeningocele and other forms of spinal
dysraphism, it is important to provide par-
ents with appropriate, realistic expectations
for their child. Delays in acquisition of gross
motor milestones by children with myelo-
meningocele have been documented across
all lesion levels, including those with minimal
or no paralysis.95,loo Although level of paraly-
sis is crucial in determining the degree of
gross motor delay, fine motor delay, and in-
dependence that can be expected, a wide vari-
ability in performance occurs within lesion
level loo Delays in gross and fine
motor skills are frequently seen in those my-
elodysplastic children with axial hypotonia,
delayed integration of primitive reflexes, and
delayed acquisition of protective responses.
These delavs in motor skills have been hv-
pothesized;o be related to associated cent;al
nervous system malformations as well as pos-
sible sequelae from meningitis or abnormal
cerebrospinal fluid pressure.
Okamoto and colleagues79and Sousa and
colleagues100have reported the ages in which
20% 50%. and 80% of children with mvelo-
meningocele at varying lesion levels are able
to achieve a variety of self-care skills. It is
important to identify not only the age at
which the child can accomplish the task, but
also the age at which he or she routinely does
the task. ShurtlefP5 has converted these data
into graphic evaluation forms that allow visu-
alization of the child's current performance
in the manner of the Denver Developmental
Screening Test.
Stature
Short stature has been well documented in
children and adults with myelomeningo-
~ e l e .s4,~85,~ 97, Short stature may be caused, in
part, by spinal deformity, reduction in limb
growth secondary to lower extremity paraple-
gia, renal insufficiency, chronic illness, early
puberty with accelerated skeletal maturation,
or nutritional compromise. Alternatively,
growth hormone deficiency has been docu-
mented in some children with neural tube
defects.86This deficiency in growth hormone
production may be secondary to associated
abnormalities of the hypothalamic pituitary
axis. Growth hormone deficiency or growth
hormone inadequacy is being increasingly
recognized and treated in the population with
myelodysplasia.
Obesity
Studies on body composition in myelomen-
ingocele have shown that these children, par-
ticularly those with thoracic to L-2 lesions,
have increased adiposity.84,93 Asher and
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM
Olson4 demonstrated obesity to be signifi-
cantly related to mobility outcomes in the
thoracic and in the upper lumbar lesion
groups. Obesity is likely secondary to a rela-
tively more sedentary existence with reduced
energy expenditure in those with greater de-
grees of paralysis. Arm-span measurement is
a helpful alternative measurement of linear
growth for determination of stature-to-weight
ratio. Skin-fold measurements may also be
followed serially for estimation of fat stores.
Dietary management is essential for achieve-
ment of weight reduction or stabilization.
Liptak and colleague^^^ compared the inci-
dence of obesity in those children with mye-
lomeningocele in a standing program versus
the incidence in those using wheelchairs and
found that upright mobility provided no pro-
tective effect from the occurrence of obesity.
Shurtleff9%dvises a 20°/0 reduction in calcu-
lated calorie needs per kilogram for severely
paralyzed infants and a 10% to 15%reduction
for moderately paralyzed infants.
Cryptorchidism
Males with myelomeningocele, particularly
those with lesions higher than L-2, have a
high incidence of cryptor~hidism.~~, " Nor-
mally the genitofemoral nerve (arising from
the L-1 and L-2 roots) innervates the guber-
naculum, a mesenchymal branch that plays
an important role in testicular descent. An-
drogens appear to affect the gubernaculum
via the genitofemoral nerve, and experimen-
tal evidence suggests that spinal cord lesions
in males at and proximal to the L-2 level
may impair genitofemoral nerve function
and prevent transinguinal descent of the
te~tes.~~,~Wrchiopexy at 1 year of age is rec-
ommended in males with cryptorchidism to
reduce the risk of infertility and testicular
cancer.
Pubertal Progression
Females with myelodysplasia have been
documented to have an increased incidence
of precocious puberty.23,46 On the average,
females with myelomeningocele achieve Tan-
ner stages I11 to V for breast development
1 to 2 years early compared with normals.
Whether boys with myelodysplasia also have
405
an increased incidence of sexual precocity has
not been established. Hydrocephalus and
other developmental or acquired abnormali-
ties of the central nervous system in children
with myelodysplasia may be the cause of
early pubertal progression in some children.
Alternatively the role of obesity on pubertal
progression in this population has not been
fully evaluated. After the age of 4, spinal
dysraphic children experience a relative defi-
cit in lean tissue, which is compensated by
an increase in adipose tissue." The increased
adiposity in children with spinal dysraphism
may be causally related to accelerated puber-
tal progression in some children. The skeletal
maturation of many children with neural tube
defects becomes advanced relative to normals
at approximately age 9.3%ccelerated skeletal
maturation may result in decreased final ob-
tained height. Some centers have been using
gonadotropin-releasing hormone analog ther-
apy in children with spinal dysraphism to
suppress puberty and delay skeletal matura-
tion in hopes of improving the ultimate
adult stature.
Latex Allergy
An increased incidence of latex allergy has
been well documented in children with spinal
dysraphism. Latex is a habitual allergen, and
sensitization likely occurs with repeated ex-
posures to latex-containing medical products.
In spinal dysraphism, latex allergy is likely
acquired through multiple exposures with
breakdown of blood-tissue barriers. Severe la-
tex reactions are associated with increased
number of previous surgical procedures. Al-
lergy testing (with skin testing or with sero-
logic determination of latex-specific IgE by
enzyme-linked immunosorbent assay) is rec-
ommended before surgical procedures for
children with spinal dysraphism. Many rec-
ommend that all surgical procedures in spinal
dysraphism patients, from the time of birth,
be performed in a latex-free e n v i r ~ n m e n t . ~ ~
Sex Education and Reproductive
Counseling
Sexual development, sexual functioning,
and reproductive potential are important con-
cerns for adolescents with spinal dysraphism,
406 McDONALD
and these issues are frequently laden with
myth and misinformation. In comparison
with able-bodied peers, adolescents with my-
elodysplasia more frequently experience
increased social isolation, decreased self-
esteem, decreased knowledge about sex, inse-
curity about their own sex organs, and con-
cerns regarding their ability to bear children.40
These psychosexual problems provide imped-
iments to normal adolescent adiustment. Pro-
vision of accurate sex education, including
information about safe sex, is part of routine
health care maintenance for the child and
adolescent with spinal dysraphism. Many
studies document that adults with myelo-
meningocele are sexually active, desire sex,
satisfy their partners, marry, and have chil-
dren.7,l5,20, 26,56,96
The most appropriate assumption is that
women with myelomeningocele are fertile,
and many are able to experience orgasm un-
less they have other anomalies such as cloaca1
e x ~ t r o p h y .The~ ~ recurrence
,~~ risk of a neural
tube defect for a woman with myelodysplasia
is approximately 4%. Pregnant women with
myelodysplasia have an increased risk of uri-
nary tract infection, back pain, and perineal
prolapse p o s t p a r t ~ m .For
~ ~ men, fertility is
theoretically possible but must be evaluated
on an individual basis given the variable de-
grees of myelodysplasia; possibility of retro-
grade ejaculation into the bladder; and possi-
ble complications of daily clean intermittent
catheterization, which include recurrent uri-
nary tract infections, prostatic calculi, prosta-
titis, and epididymitis. Erection may or may
not be present (depending on integrity of the
S-2 to S-4 lower motor neuron segments and
pelvic parasympathetic nerves), may or may
not be coordinated with psychogenic arousal,
and may or may not be related to either or-
gasm or ejaculation.15Ejaculation is mediated
through sympathetic efferents from T-12 to L-
2 via the hypogastric nerve. The projectile
release of semen is mediated by parasympa-
thetic sacral outflow and somatic efferents.
Ability to experience erection is, of course,
not a prerequisite for the experience of sexual
intimacy or fulfillment. For those who are
unable to experience erection, however, who
wish to engage in sexual intercourse with an
identified partner, implantable penile pros-
thetic devices or vacuum tumescence devices
have been used in men with both spinal cord
injury and spinal dysraphism.
COGNITION, LANGUAGE, AND
FINE MOTOR SKILLS IN SPINAL
DYSRAPHISM
Children with myelomeningocele have
been consistently documented to have intelli-
gence below the population average but
within the normal range.32, M c L ~ n has
e~~
reported that only 9% of children with spina
bifida have an IQ less than 70, and three
quarters have IQ greater than 80. The higher
the lesion, the lower the score on full-scale
and performance IQ tests. When McLone and
associates73excluded central nervous system
infections and intracranial bleeding, both
nonshunted and shunted children had mean
IQ scores in the normal range. A pattern of
deficiencies related to visual-spatial-organiza-
tional cognitive function (e.g., perceptual mo-
tor skills and arithmetic) is common. In addi-
tion, speeded perceptual-motor processing
abilities are strongly associated with motor
lesion level with nonambulatory children per-
forming more poorly.25This is likely more a
function of the severity of the central nervous
system malformation than ambulation per se.
Mean IQ, achievement, and visual-motor inte-
gration scores for myelomeningocele and li-
pomyelomeningocele have been reported by
Friedrich and colleague^^^^ 33 and are shown
in Table 1.
Myelomeningocele children have also been
noted to have generalized deficits in both vi-
sual memory and auditory/verbal memory
skills.16,99 This appears to be related to prob-
lems with encoding/consolidation skills and
organizational/retrieval abilities.
Language of children with hydrocephalus
has been previously described in selected
cases to exhibit irrelevant content and exces-
sive chatterlo4but age-appropriate vocabulary
and grammatical construction that exceeds
the actual comprehension level. Hydroce-
phalic children with normal range intelli-
gence were shown to exhibit semantically
age-appropriate responses to linguistically
posed problems.13
Problems with upper extremity coordina-
tion, fine graphomotor dexterity, and speeded
fine motor performance have been well docu-
mented in children with spinal dysraphi~m.~~,
49, 95, loo These deficits have been demon-
strated with or without shunted hydrocepha-
lus but are more pronounced in hydrocephal-
ics. Those with thoracic and high lumbar
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM 407

Table 1. MEAN IQ, ACADEMIC ACHIEVEMENT, AND BEERY VISUAL-MOTOR INTEGRATION SCORES IN
MYELODYSPLASIA
Myelomeningocele
Lipomyelomeningocele
L-2 and S-1 and
Test Above L3-5 Below Total Group Total Group
Verbal IQ 77.3 89.4 95.4 88.6 100.4
Performance IQ 77.3 89.7 90.2 87.3 106.4
Full Scale IQ 75.7 89.2 92.1 87.2 103.4
WRAT-R Reading 102.8 112.4
WRAT-R Spelling 97.1 101.4
WRAT-R Mathematics 87.4 96.9
Beery Visual-Motor 77.6 81.7
Integration

Data from references 32 and 33.

lesions tend to show greater hand function
impairment than those with midlumbar to
sacral lesions.95Upper extremity dysfunction
may be attributable to hydrocephalus, under-
lying Chiari-related cerebellar or pyramidal
tract abnormalities, or progressive cervical
myelopathy.
Intervention strategies for the above-men-
tioned impairments include (1) develop-
mental programming focusing on provision
of structured environmental enrichment for
children with developmental delay, (2) cogni-
tive remediation of identified problems with
hopes that repetition of a set of graded tasks
in the domain of concern will generalize and
carry over to functional activities, and (3)
compensation whereby specific alternative
strategies are developed to help the child
learn to perform specific skills as indepen-
dently and efficiently as possible. Computers
are excellent vehicles for interactive learning
and for improving concepts learning, atten-
tion, and visual-spatial abilities. Fine motor
impairments can be accommodated by use of
simple switches that bypass the keyboard,
allowing the child to concentrate on specific
learning tasks rather than production of writ-
ten output.
PROVISION OF THERAPY TO
ENHANCE FUNCTION
Physical therapy and occupational therapy
are often prescribed to enhance the functional
status of children with spinal dysraphism.
Physical therapists may be thought of as
movement specialists and focus on musculo-
skeletal issues, gross motor skills, and mobil-
ity. Occupational therapists may be thought
of as functional activity specialists and focus
on fine motor skills, self-care skills, visual
perceptual skills, and provision of adaptive
equipment. Oral motor skills may be man-
aged therapeutically by both occupational
therapists and speech pathologists. Therapeu-
tic goals for physical therapy and occupa-
tional therapy that often pertain to children
with spinal dysraphism are summarized in
the following lists:
Physical Therapy Goals
Facilitate acquisition of gross motor devel-
opmental milestones
Sitting balance
Rolling
Reciprocal crawling
Pulling to stand
Improve static and dynamic standing bal-
ance
Initiate gait training to improve ambulation
with appropriate assistive devices
Facilitate acquisition of independent pres-
sure relief and transfer skills
Provide developmentally appropriate func-
tional activities and play activities to en-
hance lower extremity strength and en-
durance
Provide appropriate progressive resistive
exercises to strengthen selected lower ex-
tremity muscle groups (older children)
Provide neuromuscular electrical stimula-
tion (NMES) to increase bulk and
strength of lower extremity musculature
Monitor and maintain passive range of mo-
tion in lower extremities; instruct family
in home program
Monitor fit and repair of orthotics and mo-
bility assistive devices
408 McDONALD
Perform annual lower extremity manual
muscle testing and quantitativk strength
testing (with dynamometer) for monitor-
ing of neurologic complications
Monitor serial sensorv examination for
monitoring of neurologic complications
Teach family and child monitoring of skin
over anesthetic pressure-sensitive areas
Evaluate pathfinding in community
Assist with development of appropriate
adaptive recreation programs and adap-
tive physical education; educate and as-
sist with exploration of wheelchair sports
Occupational Therapy Goals
Facilitate acquisition of fine motor develop-
mental milestones
Grasp and prehension
Bimanual skills
Proper positioning of trunk to allow for
development of upper extremity skills
Facilitate development of oral motor skills
and independent feeding
Monitor for presence of dysphagia
Oral sensitization or desensitization tech-
niques as needed
Oral motor exercises
Prefeeding regimens
Appropriate food selection and prepara-
tion
Enhance acquisition of age-appropriate
self-care skills (feeding, dressing, toi-
leting, bathing, hygiene, and higher-
level activities of daily living)
Teach family and child transfer techniques
to toilet, tub, or shower bench
Provide developmentally appropriate func-
tional activities and play activities to
enhance proximal and distal upper ex-
tremity strength and endurance
Provide progressive resistive exercises to
strengthen upper extremities (older
children)
Monitor and maintain ~ a s s i v emotion in
I
upper extremities; instruct family in
home program
Monitor seating and positioning in wheel-
chair; address revair needs of wheel-
chair; provide adaptive equipment for
self-care skills
Perform upper extremity quantitative
strength testing (grip strength and dy-
namometer testing) and standard mea-
surements of fine motor skills (e.g.,
Jebson Taylor Hand Function Test) for
monitoring of neurologic complica-
tions
Monitor serial sensory examination for
monitoring of neurologic complica-
tions
Teach family and child monitoring of skin
over anesthetic pressure-sensitive ar-
eas
Evaluate and improve visual perceptual
skills, visual scanning, and visual mo-
tor integration
Improve computer keyboarding skills to
compensate for decreased fine motor
speed and graphomotor dexterity
Initiate disabled drivers' education and
training
Specific goals are strongly related to age,
developmental status, level of neurologic im-
pairment, and functional status.
Benefits of therapy and physical training
may be observed in a relatively short period
of time. A structured 10-week exercise pro-
gram in spina bifida children aged 8 to 13
years resulted in gains versus controls on
measures of self-concept, cardiovascular en-
durance, and isometric muscle ~trength.~ Al-
ternatively, neuromuscular electrical stimula-
tion (NMES) applied to functioning muscles
(e.g., quadriceps) by surface stimulation for
30 minutes each day over an 8-week period
has been demonstrated to result in improved
peak torque production and improvement in
functional tasks (timed walking and timed
step ascension/des~ension).~~
SCHOOL AND VOCATIONAL
INTEGRATION
School participation is a major task of child-
hood and contributes to academic, personal,
and social growth. The majority of children
with spinal dysraphism are in fully main-
streamed academic programs or main-
streamed for at least part of the day.61,72 Fed-
eral law mandates that children between ages
3 and 21, regardless of severity of disability,
be provided a free, appropriate public educa-
tion that includes an individual education
program (IEP) implemented in the least re-
strictive environment. This environment is
one in which the child can have interaction
with children without disabilities. The IEP is
reviewed by staff and parents on at least an
annual basis. Children who are mainstreamed
for part of the day may receive resource room
instruction as needed under the umbrella of
special education.
 REHABILITATION OF CHILDREN WITH SPINAL DYSRAPHISM
Local and state educational agencies are
responsible for providing children with disa-
bilities transportation and access to school;
special education; and services (if indicated)
such as speech, occupational, physical, and
recreational therapy, counseling, and nursing.
A supreme court decision has established that
children at school must be provided with as-
sistance for clean intermittent catheterization
by school personnel in a private setting and
that such needs are not adequate reasons to
exclude children from mainstreamed educa-
tion.
Questions and concerns from educators
usually focus on (1) health maintenance
needs; (2) physical status and transportation
needs; (3) status of vision and hearing; (4)
adaptive functioning and need for therapy;
(5) endurance; (6) adjustment issues and need
for special counseling or guidance; (7) prog-
nostic expectations relevant to developing an
educational program; (8) identification of hos-
pital liaison personnel for assistance with
problem solving; and (9) information regard-
ing future plans, evaluations, and treatments
likely to interfere with school attendance.
Estimates of employment in myelomenin-
gocele vary from 17% to 74%.95McAndrew'j6
found that the intellectual ability of spina bi-
fida patients was more important than physi-
cal disability for predicting successful em-
ployment. Referral to the State Vocational
Rehabilitation program is important on entry
to high school. These programs carefully con-
sider an individual's specific disability to help
recommend vocational possibilities; often
perform testing to identify vocational inter-
ests and aptitudes; provide funding for job
training, higher education, transportation,
and adaptive equipment to facilitate employ-
ment; and assist with identification of em-
ployment opportunities.
Work-study experiences beginning in high
school may provide opportunities for the dis-
abled adolescent and young adult to explore
career possibilities, integrate into the work
world, develop a realistic self-appraisal and
enhancement of skills, and develop work
habits.
ADJUSTMENT TO DISABILITY AND
TRANSITION FROM ADOLESCENCE
TO ADULTHOOD
Families of disabled children experience
different stresses that require varied coping
409
strategies as they move through life's transi-
tions. During early childhood, problems re-
lated to physical management are a priority.
The emphasis during school-age years typi-
cally shifts to school programming, ongoing
appraisal of the child's development, and
concerns regarding limited peer involvement.
The adolescent has specific issues pertaining
to body image and personal identity; estab-
lishment of control and indevendence from
parents; acceptance of the "permanence" of
disability; and issues regarding sexuality,
peer relations, and social is~lation.~,
9Voung
adults with congenital disabilities are often
continuing to work through issues of adoles-
cence. This often leads to delav and difficultv
i J
moving through the typical issues of young
adulthood, such as decision making about
future life goals and occupational choices, ex-
vloration and consolidaiion of friendshivs.
lchoice of a possible mate, establishmentLof
adult relations with parents and other family
members. and achievement of financial inde-
pendence. Ongoing psychosocial support of
patient and family is particularly important
during this transition, and it is essential that
interd<sciplinary management continue for
the young adult with spinal dysraphism.
SUMMARY
Tremendous advances have occurred over
the past several decades in the comprehen-
sive management of spinal dysraphism. Mul-
tiple specialists from varied surgical, medical,
and allied health wrofessions contribute to the
rehabilitation m;nagement of children with
spinal dysraphism. Rehabilitation goals and
treatment strategies" have been vresented
I
in
the contexts of multiple functional outcome
domains of critical importance to quality of
life. In addition, the child with spinal dys-
r a ~ h i s moften has uniaue health care mainte-
nance issues that require careful monitoring
within a coordinated, interdisciplinary clinic
setting. Prevention of neurologic, musculo-
skeletal, urologic, and medical complications
is necessary for the child with spinal dysraph-
ism to realize his or her maximum functional
potential. A comprehensive rehabilitation
program provides sufficient training, educa-
tion, and long-term monitoring to enable the
child not only to achieve self-sufficiency, but
also to maintain an optimal level of function
throughout life.
410 McDONALD
ACKNOWLEDGMENT
The author wishes to thank Tammy Vaughan for her
assistance with manuscript preparation.
References
1. Agre JC, Findley TW, McNally MC, et al: Physical
activity in children with myelomeningocele. Arch
Phys Med Rehabil 68:372-377, 1987
2. Aitkens S, Lord J, Bernauer E, et al: Relationship of
manual muscle testing to objective strength mea-
surements. Muscle Nerve 12:173-177, 1989
3. Andrade CK, Garben M: Changes in self-concept,
cardiovascular endurance and muscular strength of
children with spina bifida aged 8 to 13 years in
response to a 10-week physical activity programme:
A pilot study. Child Care Health Dev 17:183-196,
1991
4. Asher M, Olson J: Factors affecting the ambulatory
status of patients with spina bifida cystica. J Bone
Joint Surg 65A.350-356, 1983
5. Badell A: Myelodysplasia. In Molnar GE (ed): Pedi-
atric Rehabilitation, ed 2. Baltimore, Williams & Wil-
kins, 1992
6. Banta JV,Casey JM, Bedell L, et al: Long-term am-
bulation in spina bifida. Dev Med Child Neurol
25:110, 1983
7. Barden BA, Meyer DC, Stelling FH: Myelo-
dysplasia-fate of those followed for twenty years
or more. J Bone Joint Surg 57A:643-647, 1975
8. Beasley WC: Quantitative muscle testing: Principles
and applications to research and clinical services.
Arch Phys Med Rehabil42:398425,1961
9. Blum RW, Resnick MD, Nelson R, et al: Family and
peer issues among adolescents with spina bifida and
cerebral palsy. Pediatrics 88:280-285, 1991
10. Butler C: Effects of powered mobility on self-initi-
ated behaviors of very young children with locomo-
tor disability. Dev Med Child Neurol 28:325-332,
1986
11. Butler C: Augmentative mobility: Why we do it?
Phys Med Rehabil Clin North Am 2:801-813, 1991
12. Butler C, Okamoto GA, McKay TM: Motorized
wheelchair driving by disabled children. Arch Phys
Med Rehabil65:95-97,1984
13. Byrne K, Abbeduto L, Brooks P: The language of
children with spina bifida and hydrocephalus:
Meeting tasks demands and mastering syntax. J
Speech Hear Disord 55:118-123, 1990
14. Charney E, Melchionni JB, Smith DR: Community
ambulation by children with rnyelomeningocele and
high-level paralysis. J Pediatr Orthop 11:579-582,
1991
15. Comarr AE: Neurological disturbances of sexual
function among patients with myelodysplasia. In
McLaurin RL (ed): Myelomeningocele. Orlando, FL,
Grune & Stratton, 1977, pp 797-808
16. Cull C, Wyke MA: Memory function of children
with spina bifida and shunted hydrocephalus. Dev
Med Child Neurol 26:177-183, 1984
17. Curtis BH, Brightman E: Spina bifida: A follow-up
of ninety cases. Conn Med 26:145-150, 1962
18. Davidson HM: The Isocentric reciprocating gait or-
thosis. Assoc Prosthet Orthot J 1:12-15, 1994
19. De Souza LJ, Carroll N: Ambulation of the braced
myelorneningocele patient. J Bone Joint Surg
58A:1112-1119, 1976
20. Dorner S: Adolescents with spina bifida: How they
see their situation. Arch Dis Child 51:539-544, 1976
21. Douglas R, Larson PF, D'Ambrosia RD, et al: The
LSU Reciprocation-Gait orthosis. Orthopaedics
6:834-839, 1983
22. Effgen SK, Brown DA: Long-term stability of hand-
held dynamometric measurements in children who
have rnyelomeningocele. Phys Ther 72:458465,1992
23. Elias ER, Sedeghi-Nejad A: Precocious puberty in
girls with myelodysplasia. Pediatrics 93:521-522,
1994
24. Ellsworth PJ, Merguerian PA, Klein RB, et al: Evalu-
ation and risk factors of latex allergy in spina bifida
patients: Is it preventable? J Urol 150:691-693, 1993
25. Evaggelinou C, Drowatzky JN: Timing responses of
children with spina bifida having varying ambula-
tory abilities. Percept Motor Skills 73:919-928, 1991
26. Evans K, Hickman V, Carter CD: Hardship and
social status of adults with spina bifida cystica. Br J
Prev Soc Med 28:85-92, 1974
27. Feiwell E, Sakai D, Blatt T: The effect of hip reduc-
tion on function in patients with myelomeningocele:
Potential gains and hazards of surgical treatment. J
Bone Joint Surg 60k169-173, 1978
28. Findley TW, Agre JC, Habeck RV, et al: Ambulation
in the adolescent with myelomeningocele I: Early
childhood predictors. Arch Phys Med Rehabil
68:518-522, 1987
29. Flandry F, Burke S, Roberts JM, et al: Functional
ambulation in myelodysplasia: The effect of orthotic
selection on physical and physiologic performance.
J Pediatr Orthop 6:661-665, 1986
30. Franks CA: A comparison of the effects of crutch
walking and wheelchair propulsions on three mea-
sures of school performance in students with myelo-
meningocele. Master's thesis, Hahnemann Univer-
sity, Philadelphia, 1989
31. Fraser EK, Menelaus MB: The management of tibia1
torsion in patients with spina bifida. J Bone Joint
Surg 75B:495497, 1993
32. Friedrich WN, Lovejoy MC, Shaffer J, et al: Cogni-
tive abilities and achievement status of children
with myelomeningocele: A contemporary sample. J
Pediatr Psycho1 16:423428, 1991
33. Friedrich WN, Shurtleff DB, Shaffer J: Cognitive
abilities and lipomyelomeningocele. Psych Rep
73:467470, 1993
34. Gaff JE, Robinson JM, Parker PM: The walking abil-
ity of 14- to 17-year-old teenagers with spina
bifida-a physiotherapy study. Physiotherapy
70:473474, 1984
35. Greene SA, Frank M, Zaehmann M, et al: Growth
and sexual development in children with myelo-
meningocele. Eur J Pediatr 144:146-148, 1985
36. Greene WB, Carter MD, DeMasi RA, et al: Bone
mineral density in myelomeningocele: Effect of
growth and other factors. Dev Med Child Neurol
64(suppl):18, 1991
37. Grimm RA: Hand function and tactile perception in
a sample of children with rnyelomeningocele. Am J
Occup Ther 30:234-240, 1976
38. Guidera KJ, Smith S, Raney E, et al: Use of the
reciprocating gait orthosis in myelodysplasia. J Ped-
iatr Orthop 13:341-348, 1993
39. Harris MB, Banta JV: Cost of skin care in the myelo-
 REHABILITATION (IF CHILDREN WITH SPINAL DYSRAPHISM
meningocele population. J Pediatr Orthop 10:355-
361, 1990
40. Hayden PW, Davenport LH, Campbell MM: Ado-
lescents with myelodysplasia: Impact of physical
disability on emotional maturation. Pediatrics
64:53-59, 1979
41. Hayes-Allen MS: Obesity and short stature in chil-
dren with myelomeningocele. Dev Med Child Neu-
rol 14(suppl 22):59-64, 1972
42. Hays RM, Jordan RA, McLaughlin JF, et al: Central
ventilatory dysfunction in myelodysplasia: An inde-
pendent determinant of survival. Dev Med Child
Neurol 31:366-370, 1989
43. Hoffer MM, Feiwell E, Perry R, et al: Functional
ambulation in patients with myelomeningocele. J
Bone Joint Surg 55A:137-148, 1973
44. Huff CW, Ramsey PL: Myelodysplasia: The influ-
ence of the quadriceps and hip abductor muscles
on ambulatory function and stability of the hip. J
Bone Joint Surg 60A:432443, 1978
45. Hunt GM: Spina bifida: Implications for 100 chil-
dren at school. Dev Med Child Neurol 23:160-172,
1981
46. Hunt GM: Open spina bifida: Outcome for a com-
plete cohort treated unselectively and followed into
adulthood. Dev Med Child Neurol 32:108-118,1990
47. Hutson JM, Beasley SW, Bryan AD: Cryptorchidism
in spina bifida and spinal cord transection: A clue
to the mechanism of transinguinal descent of the
testis. J Pediatr Surg 23:275-277, 1988
48. Hutson JM, Donahoe PK: The hormonal control of
testicular descent. Endocr Rev 7270-283, 1986
49. Jansen J, Taudorf K, Pederson H, et al: Upper ex-
tremity function in spina bifida. Childs Nerv Syst
767-71, 1991
50. Karmel-Ross K, Cooperman DR, Van Doren CL: The
effect of electrical stimulation on quadriceps femoris
muscle torque in children with spina bifida. Phys
Ther 72:723-730, 1992
51. Kaufman BA, Terbrock A, Winters N, et al: Dis-
banding a multidisciplinary clinic: The effects on the
health care of myelomeningocele patients. Pediatr
Neurosurg 21:3644, 1994
52. King JC, Currie DM, Wright E: Bowel training in
spina bifida: Importance of education, patient com-
pliance, age and reflexes. Arch Phys Med Rehabil
75:243-247, 1994
53. Kropp KA, Voeller KKS: Cryptorchidism in menin-
gomyelocele. J Pediatr 99:110-113, 1981
54. Landry SH, Robinson SS, Copeland D, et al: Goal-
directed behavior and perception of self-competence
in children with spina bifida. J Pediatr Psycho1
18:389-396, 1993
55. Latcha CM, Freeling MC, Powell NJ: A comparison
of the grip strength of children with myelomeningo-
cele to that of children without disability. Am J
Occup Ther 47498-502,1993
56. Laurence KM, Beresford A: Continence, friends,
marriage, and children in 51 adults with spina bi-
fida. Dev Med Child Neurol 17(suppl 35):123-128,
1975
57. Letts RM, Fulford R, Hobson DA: Mobility aids for
the paraplegic child. J Bone Joint Surg 5 8 ~ : 3 8 4 1 ,
1976
58. Liptak GS, Reveli GM: Management of bowel dys-
function in children with spinal cord disease on
injury by means of the enema continence catheter. J
Pediatr 120:190-194, 1992
411
59. Liptak GS, Shurtleff DB, Bloss JW, et al: Mobility
aids for children with high-level myelomeningocele:
Parapodiuln versus wheelchair. Dev Med Child
Neurol 34:787-796, 1992
60. Loenig-Baucke V, Desch L, Wolraich M: Biofeed-
back training for patients with myelomeningocele
and fecal incontinence. Dev Med Child Neurol
30:781-790, 1988
61. Lord J, Varzos N, Behrman B, et al: Implications of
mainstream classrooms for adolescents with spina
bifida. Dev Med Child Neurol 32:20-29, 1990
62. Maynard MJ, Weiner LS, Burke SW: Neuropathic
foot ulceration in patients with myelodysplasia. J
Pediatr Orthop 12:786-788, 1992
63. Mazur JM, Shurtleff D, Menelaus M, et al: Ortho-
paedic management of high-level spina bifida: Early
walking compared with early use of a wheelchair. J
Bone Joint Surg 71A:56-61, 1989
64. Mazur JM, Sienko-Thomas S, Wright N, et al:
Swing-through vs. reciprocating gait patterns in pa-
tients with thoracic-level spina bifida. Z Kinderchir
45(suppl 1):23-25, 1990
65. Mazur JJ, Stillwell A, Menelaus M: The significance
of spasticity in the upper and lower limbs in myelo-
meningocele. J Bone Joint Surg 68B:213-217, 1986
66. McAndrew I: Adolescent and young people with
spina bifida. Dev Med Child Neurol 21:619-629,
1979
67. McCall RE, Schmidt T: Clinical experience with the
reciprocal gait orthosis in myelodysplasia. J Pediatr
Orthop 6:157-167, 1986
68. McDonald CM, Abresch RT, Aitkens SG, et al: Pro-
files of neuromuscular diseases: Duchenne muscular
dystrophy. Am J Phys Med Rehabil, in press
69. McDonald CM, Jaffe KM, Mosca VS, et al: Ambula-
tory outcome of children with myelomeningocele:
Effect of lower extremity muscle strength. Dev Med
Child Neurol33:482-490, 1991
70. McDonald CM, Jaffe KM, Shurtleff DB: Assessment
of muscle strength in children with meningomyelo-
cele: Accuracy and stability of measurements over
time. Arch Phys Med Rehabil 672355-861, 1986
71. McDonald CM, Jaffe KM, Shurtleff DB, et al: Modi-
fications to the traditional description of neuroseg-
mental innervation in myelomeningocele. Dev Med
Child Neurol33:473481, 1991
72. McLone DG: Continuing concepts in the manage-
ment of spina bifida. Pediatr Neurosurg 18:254-
256, 1992
73. McLone DG, Czyzewsky D, Raimondi AJ, et al:
Central nervous system infections as a limiting fac-
tor in the intelligence of children with myelomenin-
gocele. Pediatrics 70:338-342, 1982
74. Motloch W: The parapodium: An orthotic device for
neuromuscular disorders. Artificial Limbs 15:36-47,
1971
75. Motloch W: Accent on inventions: The birth of the
RGO. Orthotics Prosthetics Almanac 49-50, 1994
76. Motlock WM, Elliott J: Fitting and training children
with swivel walkers. Artificial Limbs 10:27-38, 1966
77. Mustoe TA, Cutler NR, Allman RM, et al: A phase
I1 study to evaluate recombinant platelet-derived
growth factor-BB in the treatment of stage 3 and 4
pressure ulcers. Arch Surg 129:213-219, 1994
78. Okamoto GA, Lamers JV, Shurtleff DB: Skin break-
down in patients with myelomeningocele. Arch
Phys Med Rehabil 64:20-23, 1983
79. Okamoto GA, Sousa J, Telzrow RW, et al: Toileting
412 McDONALD
skills in children with myelomeningocele: Rates of
learning. Arch Phys Med Rehabil 65:182-185, 1984
80. Olness K, McPharland FA, Piper J: Biofeedback: A
new modality in the management of children with
fecal soiling. J Pediatr 96:505-509, 1980
81. Pollack IF, Pang D, Kocoshis S, et al: Neurogenic
dysphagia resulting from Chiari malformations.
Neurosurgery 30:709-719, 1992
82. Putnam PE, Orenstein SR, Pang D, et al: Cricopha-
ryngeal dysfunction associated with Chiari malfor-
mations. Pediatrics 89:871-876, 1992
83. Radke J, Gosky GA: Hearing and speech screening
in hydrocephalus myelodysplasia population. Spina
Bifida Ther 3:25, 1981
84. Roberts D, Shepherd RW, Shepherd K: Anthropom-
etry and obesity in myelomeningocele. J Paediatr
Child Health 2783-90, 1991
85. Rosenblum MF, Finegold DN, Chamey EB: Assess-
ment of stature of children with myelomeningocele
and usefulness of arm-span measurements. Dev
Med Child Neurol25:338-342, 1983
86. Rotenstein D, Reigel DH, Flom LL: Growth hor-
mone treatment accelerates growth of short children
with neural tube defects. J Pediatr 115:417420,1989
87. Samuelsson L, Skoog M: Ambulation in patients
with myelomeningocele: A multivariate statistical
analysis. J Pediatr Orthop 8:569-575, 1988
88. Sand PL, Taylor N, Hill M, et al: Hand function in
children with myelomeningocele. Am J Occup Ther
28:87-90,1974
89. Schopler SA, Menelaus MB: Significance of the
strength of quadriceps muscles in children with my-
elomeningocele. J Pediatr Orthop 7507-512,1987
90. Shandling B, Gilmour RF: The enema continence
catheter in spina bifida: Successful bowel manage-
ment. J Pediatr Surg 22:271-273, 1987
91. Sharrard WJW: The segmental innervation of the
lower limb muscles in man. Ann R Coil Surg 35:106-
122, 1964
92. Shepherd K, Hickstein R, Shepherd R: Neurogenic
fecal incontinence in children with spina bifida: Rec-
<osphincteric responses and evaluation of a physio-
logical rationale for management, including biofeed-
back conditioning. Aust Paediatr J 19:97-99, 1983
93. Shepherd K, Roberts D, Golding S, et al: Body com-
position in myelomeningocele. Am J Clin Nutr
53:l-6, 1991
94. Sherk HH, Uppal GS, Lane G, el al: Treatment ver-
sus non-treatment of hip dislocations in ambulatory
patients with myelomeningocele. Dev Med Child
Neurol33:491494, 1991
95. Shurtleff DB (ed): Myelodysplasias and Exstrophies:
Significance, Prevention and Treatment. Orlando,
FL, Grune & Stratton, 1986
96. Shurtleff DB, Hayden PW, Chapman WH, et al:
Myelodysplasia: Problems of long-term survival
and social function. West J Med 122:199-205, 1975
97. Shurtleff DB, Lamers J, Goiney T, et al: Are myelo-
dysplastic children fat? Anthropometric measures:
A preliminary report. Spina Bifida Ther 41-21,1982
98. Shurtleff DB, Souza JC: The adolescent with myelo-
dysplasia: Development, achievement, sex, and de-
terioration. Del Med J 49:631-638, 1977
99. Snow JH: Memory functions for children with spina
bifida. Assessment in Rehabilitation and Exception-
ality. Pediatrics 1:20-27, 1994
100. Sousa JC, Telzrow RW, Holm RA, et al: Develop-
mental guidelines for children with myelodysplasia.
J Am Ther Assoc 63:21-29, 1983
101. Stillwell A, Menelaus MB: Walking ability in mature
patients with spina bifida. J Pedgtr 0rthop 3:184-
190, 1983
102. Stolov WC, Hays RM: Evaluation of the patient. In
Kotke JF, Lehmann JF, Stillwell GK (eds): Krusen's
Handbook of Physical Medicine and Rehabilitation.
Philadelphia, FA Davis, 1990, pp 1-19
103. Swank M, Dias L: Myelomeningocele: A review of
the orthopaedic aspects of 206 patients treated from
birth with no selection criteria. Dev Med Child Neu-
rol34:1047-1052, 1992
104. Swisher L, Pinscher E: The language characteristics
of hyperverbal hydrocephalic children. Dev Med
Child Neurol 13:746, 1981
105. Taylor N, Sand PL, Jebson RH: Evaluation of hand
function in children. Arch Phys Med Rehabil 54:
129-135, 1973
106. Telsrow RW, Campos JJ, Shepard A, et al: Spatial
understanding in infants with motor handicaps. In
Jaffe KM (ed): Childhood Powered Mobility: Devel-
opmental, Technical and Clinical Perspectives.
Washington, DC, RESNA, 1987, p 62
107. Tew B, Laurence KM, Jenkins V: Factors affecting
employability among young adults with spina bi-
fida and hydrocephalus. Z Kinderchir 45(suppl 1):
34-36, 1990
108. Thompson NM, Fletcher JM, Chapieski L, et al: Cog-
nitive and motor abilities in preschool hydrocephal-
ics. J Clin Exp Neuropsych B:245-258,1991
109. Turner A: Hand function in children with myelo-
meningocele. J Bone Joint Surg 67B:268-272,1985
110. Wald N: Biofeedback for neurogenic fecal conti-
nence: Rectal sensation is a determinant of outcome.
J Pediatr Gastroenter Nutr 2:302-306, 1983
111. Willis KE, Holmbeck GN, Dillon K, et al: Intelli-
gence and achievement in children with myelomen-
ingocele. J Pediatr Psycho1 15:161-176, 1990
112. Wood JM, Evans PE, Schallreuter KU, et al: A multi-
center study of direct current for healing of chronic
stage I1 and stage ITI decubitus ulcers. Arch Derma-
to1 129:999-1009, 1993
113. Wright JG, Menelaus MB, Broughton NS, et al: Nat-
ural history of knee contractures in myelomeningo-
cele. J Pediatr Orthop 11:725-730,1991
114. Yngve DA, Douglas R, Roberts JM: The reciprocat-
ing gait orthosis in myelomeningocele. J Pediatr Or-
thop 4:304-310, 1984
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Craig M. McDonald, MD
Department of Physical Medicine and Rehabilitation
University of California, Davis Medical Center
4301 X Street, Room 2030
Sacramento, CA 95817