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Rehabilitation and Medical Management of the Adult with Spina Bifida

Article in American journal of physical medicine & rehabilitation / Association of Academic Physiatrists · November 2008
DOI: 10.1097/PHM.0b013e31818de070 · Source: PubMed

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Authors:
Brad E. Dicianno, MD
Brad G. Kurowski, MD Spina Bifida
Jennifer Marie J. Yang, MD
Michael B. Chancellor, MD
Ghassan K. Bejjani, MD
Andrea D. Fairman, MOT, OTR/L
Nancy Lewis, RN
Jennifer Sotirake, RD, LDN
LITERATURE REVIEW

Affiliations:
From the Adult Outpatient Spina
Bifida Clinic (BED, NL, JS), Rehabilitation and Medical
Department of Physical Medicine and
Rehabilitation (BED, BGK, JMJY, NL),
University of Pittsburgh Medical
Management of the Adult
Center (UPMC), Pittsburgh,
Pennsylvania; Human Engineering
with Spina Bifida
Research Laboratories, VA Pittsburgh
Healthcare System, Pittsburgh,
Pennsylvania (BED, JMJY);
Department of Urology, William ABSTRACT
Beaumont Hospital, Royal Oak,
Michigan (MBC); Department of Dicianno BE, Kurowski BG, Yang JMJ, Chancellor MB, Bejjani GK, Fairman AD,
Neurosurgery, UPMC Presybterian Lewis N, Sotirake J: Rehabilitation and medical management of the adult with
Shadyside, Shadyside Campus, spina bifida. Am J Phys Med Rehabil 2008;87:1026 –1050.
Pittsburgh, Pennsylvania (GKB);
Spina Bifida Association of Western As the life expectancy of individuals with spina bifida increases, a lifelong need for
PA, Pittsburgh, Pennsylvania (ADF); management of many health issues in a rehabilitation setting has emerged in
Department of Rehabilitation Science recent years. Physiatrists, in consultation with a variety of adult specialists, are
and Technology, University of
Pittsburgh, Pittsburgh, Pennsylvania particularly well suited to manage the common musculoskeletal, skin, bowel,
(BED, ADF); and Department of bladder, renal, neurological, and other issues that arise in the adult population.
Nutrition, UPMC, Pittsburgh, This article reviews the last 20 yrs of literature pertinent to the rehabilitative care
Pennsylvania (JS). of this population, summarizes current evidence-based practice, and identifies key
areas in which scientific evidence is lacking and future research is needed.
Correspondence:
Key Words: Adult, Myelomeningocele, Hydrocephalus, Rehabilitation, Review, Tethered
All correspondence and requests for
reprints should be addressed to Brad Cord Syndrome, Transitional Care
E. Dicianno, MD, Human
Engineering Research Laboratories,
VA Pittsburgh Healthcare System,
7180 Highland Drive, Building 4, 2nd
Floor East, 151R1-H, Pittsburgh, PA
15206.
A dvancements in medical science have enabled individuals with spina bifida
(SB) to live longer now than ever before.1–5 This increase in life expectancy
necessitates health care extending into adulthood. Before 1975, death of infants
Disclosures: with myelomeningocele significantly impacted survival rates.6,7 Today, at least
There was no outside funding for this 75– 85% of children born with SB are expected to reach their early adult
manuscript. years.3,7 However, the life expectancy of those with SB cannot be accurately
determined from the current data available.8,9
0894-9115/08/8712-01026/0
The growing adult SB population has prompted various studies that elucidate
American Journal of Physical
Medicine & Rehabilitation
medical and rehabilitative issues pertinent to this population.2– 4,10 –24 They span a
Copyright © 2008 by Lippincott wide range of domains, including neurological, neurosurgical, cognitive, psy-
Williams & Wilkins chosocial, renal/urological, bowel management, mobility, musculoskeletal/or-
thopedic, sexual function, skin, and others.2– 4,10 –24 Renal, cardiac, and respi-
DOI: 10.1097/PHM.0b013e31818de070 ratory complications have been identified as frequent causes of death.9
Optimization of the medical and rehabilitation management of these secondary
health conditions is integral to reducing mortality, morbidity, and impairment
to improve quality of life in the adult with SB.2,12,14
Secondary complications associated with SB first occur in childhood, and
excellent initial management of these issues is essential for the transition into
adulthood.25–27 A coordinated interdisciplinary team approach to management
has been shown to be effective in the care of children with SB; however, this type

1026 Am. J. Phys. Med. Rehabil. ● Vol. 87, No. 12

of approach does not always extend into adult if their subject population included both adult
care.28 –31 In general, there is a lack of comprehen- and younger age groups.
sive and lifelong care available to the adult with
SB.32 Multidisciplinary clinics may not survive be-
RESULTS
cause they typically face many challenges, includ-
ing program structure, personnel, and financial Transition to Adult Care
support issues.33 In fact, at the time of this publi- Binks et al.28 identified five key elements nec-
cation, there are currently only five clinics in the essary for the successful transition of care for those
United States devoted solely to the care of the adult with cerebral palsy and SB. These included early
with SB.34 As those with SB age, they encounter a preparation/education of the individual and family,
variety of medical, cognitive, and psychosocial issues flexible timing of the transition, introduction to the
that physiatrists are particularly well suited to man- transition clinic, interested adult-center providers,
age. Regardless of whether a comprehensive clinic and a coordinated transfer of care approach between
exists, the physiatrist may be able to coordinate a the individual, family, pediatric primary care physi-
team of specialists needed in the care of the adult cians, and adult specialist.28 They also identified five
with SB. barriers, including child healthcare providers refus-
In this article, we will discuss the current state ing to “let go,” parents refusing to “let go,” reluctance
of the literature on the rehabilitation and medical to leave a family-centered care paradigm, and adult
management of the adult with SB and ways that center care providers having limited knowledge or
physiatrists can reduce morbidity and maximize limited interest in caring for these individuals.28
function for these individuals. Additionally, we Young adults with SB often have unmet health
highlight where there are gaps in treatment and needs.35 Young et al.29 evaluated the transition of
management protocols so areas of future research children with cerebral palsy, SB, and acquired
can be identified. brain injury to adult-centered care and found that
adults with SB reported the lowest health scores of
the three groups. In the absence of coordinated
METHODS care, young adults with physical disabilities and,
We performed a literature search of all publi- specifically, individuals with SB often fail to receive
cations dated January 1, 1988, to May 31, 2008, regular hospital care and their original diagnoses
using the search terms of “spina bifida or myelo- and prescriptions are not regularly reviewed.36,37
meningocele” in PubMed. We set the following Adult-centered hospitals are the primary facilities
limits: publication date from 1988 to May 31, 2008, in the United States where those with a history of
English, and all adult (19⫹ yrs). This initial search congenital disabilities are hospitalized when they
yielded 1425 results. These were then reviewed, become ill.38 Admission rates are nine times higher than
and manuscripts that primarily focused on the that of the general population, and 76% do not have or
rehabilitation, medical management, and out- cannot identify a primary care provider.39 This is
comes related to adults with SB or myelomeningo- alarming especially since Kinsman and Doehring40
cele were included, whereas articles that focused demonstrated that 47% of the admissions, older
primarily on children, focused primarily on spina than 11 yrs, to one acute care hospital involving
bifida occulta, were case studies or small case re- adults with SB were secondary to preventable con-
ports, or focused primarily on surgical techniques ditions. Furthermore, adults with SB have medical
were excluded. The search yielded 267 articles that expenditures 3– 6 times greater than adults with-
were included. These were then reviewed, and 85 out SB.41 Lifelong integrated care for individuals
additional citations were added based on searching with SB by a medical team is, therefore, highly
through pertinent reference lists of the included recommended.42 The nurse coordinator’s role is
manuscripts and by reviewing other relevant liter- critical to coordinating transitional and long-term
ature when there were gaps of information noted. care.43
The total number of references included was 352. It is crucial to involve families in the transition
For the purposes of this article, we use “spina process.44 – 47 The transition to autonomy in health
bifida” to refer primarily to “myelomeningocele” care should begin as early as possible.48 Significant
rather than meningocele or spina bifida occulta; difficulties arise for the aging parents who are
however, in some cited references, it is unclear providing continued care,49 especially since auton-
whether the use of spina bifida was inclusive of omy in activities of daily living, self-care, and mo-
other forms of open or occult dysraphism. We use bility tend to worsen over time.50 –52 Parents feel
“adult” to refer primarily to individuals aged 19 yrs their child’s needs for transportation increase with
and older and “young adult” to refer to those age.53 Many adults with SB remain dependent on
aged 19 –21 yrs. However, to include all impor- their parents for management of incontinence and
tant studies on adults, we did not exclude studies skin care.54 Discussions about continued care after

December 2008 Rehabilitation and Management of Spina Bifida 1027

 the aged parent is no longer able to be a caregiver with SB. About 8% of women with SB meet criteria
are important. for eating disorders,69 and if diagnosed properly,
these disorders respond well to multidisciplinary
Psychosocial and Quality of Life intervention.70 Future research should focus on
the prevalence and outcomes regarding manage-
The family and social environments play an
ment strategies of depression, anxiety, and other
integral role in the future psychosocial develop-
mental disorders in the adult SB population.
ment and quality of life of young adults with SB.
Sawin et al.55 demonstrated that adolescent stress, Education and Employment
mother’s education, parent-family satisfaction, and Educational achievement is decreased in ado-
adolescent hope are key factors for quality of life in lescents and adults with childhood disabilities.71
adolescents with SB. The future expectations of There are limited data on educational outcomes in
adolescents and parents frequently differ with re- SB. About 85% successfully graduate high school,3
spect to school, job, athletics, behavior, attractive- whereas successful college attendance is only
ness, and romantic appeal.46 Social support and 14.6%.72 Lower intelligence was the main predic-
parental hope are more strongly associated with tor of the need for special secondary education, but
self-worth and health-related quality of life than gen- hydrocephalus and wheelchair dependence were
der, age, diagnosis, or physical impairments.56,57 Per- also significant variables.73 Performance on intel-
ceived health-related quality of life of young adults ligence quotient (IQ) testing has been correlated
with SB is lower, especially within the physical func- with ventricle size and the level of lesion or highest
tioning domain, compared with a population without open vertebral arch at birth.74 –76 In the absence of
a disability, as measured using the Short-form Health hydrocephalus, young adults with SB had similar
Survey.58 outcomes compared with the general population in
When compared with age-matched able-bodied their attendance of regular vs. special secondary
comparison subjects, adolescents and young adults with education,73 and cognitive functioning measured
SB were at greater risk of depressed mood, low self- with neuropsychological testing was relatively un-
worth, and suicidal ideation.59 Social and peer re- impaired.77,78 Other impairments that affect edu-
lationships and social “norms” play a significant role cation include decreased fine motor coordination;
in the development of positive or negative disability motor timing impairments; decreased speed of in-
identity.60,61 The highest proportion of life dissatis- formation processing; impaired executive function;
faction was found for financial situation, partner- decreased prospective/working memory; poor com-
ship relations, and sex life.62 Involvement in leisure putation speed and accuracy; decreased problem
activities has a positive effect on mental and phys- solving; and a slow learning curve.73,79 – 88 Motor
ical health, enjoyment, opportunities to develop in- skill deficits could be improved with practice, especially
creased self-esteem, and opportunities to build positive speed and accuracy.89 Additionally, Huber-Okrainec
social relationships.63 Self-concept, which is lower in et al.90 demonstrated that individuals with SB and
adolescents with SB compared with those without dis- hydrocephalus compared with controls had an in-
abilities, and self-efficacy, the belief in one’s ability to creased number of motor speech deficits, including
perform a specific task, are predictors of indepen- dysarthria, impaired fluency, and impaired rate of
dence.64 Those succeeding at college and participat- speech.
ing in recreation, sport activities, and other aspects of Individuals with childhood disabilities and those
young adult living report a good health-related qual- specifically with SB are underemployed and not consis-
ity of life, but they report concerns about the number tently living independently as adults.71,91 Studies have
of secondary health conditions they are experienc- shown that 29–33% of adults with SB were in compet-
ing.65 Because people with motor disabilities are itive employment, 19–29% were in sheltered/supported
known to have fewer opportunities to participate in employment, and 25–32% were unemployed or never
social life and have limited opportunities to benefit employed.72,92 Higher cognitive status, specifically exec-
from those interactions,66 there needs to be a com- utive functioning and memory, have been positively
bined effort by healthcare professionals, the individ- correlated with everyday physical activity and functional
ual, and parents and parent groups to reduce social independence in adolescents and young adults with
isolation.67 Perceived family encouragement of inde- SB.79,93 Thus, more functional ambulatory status, in-
pendence is positively associated with achievement as creased independence in daily activities, higher educa-
a young adult.68 tional level, and higher intelligence level increase the
The physiatrist can play a key role in encour- probability of work participation.72,94,95 Specifically,
aging individuals to participate in community ac- Loomis et al.95 demonstrated that paid employment was
tivities that will enhance function and improve related to various basic skills, including academics, ver-
their quality of life. Clinical screening for depres- bal memory, attention/concentration, and adaptive be-
sion should be done on a regular basis in the adult havior, whereas the skill level of employment was

1028 Dicianno et al. Am. J. Phys. Med. Rehabil. ● Vol. 87, No. 12
associated with intelligence. Neuropsychological impair- using wheelchairs, can improve education and em-
ments, particularly in those with concomitant hydro- ployment opportunities.
cephalus, may result in difficulty with jobs or tasks that
require multitasking.96 Hetherington et al.97 demon- Ambulation and Mobility
strated a negative association between the number of
Mobility is an important determinant of qual-
shunt revisions and occupational status.97 Those with
ity of life for those with SB.107 It is integral to
revisions after the age of 2 yrs were more likely to have
independence, activities of daily living, employment,
lower overall achievement and more symptomatic epi-
community/leisure activities, and overall life satisfac-
sodes of raised intracranial pressure.98,99 Interestingly,
tion.106 Yet, adults with SB are hypoactive compared
one study did not find a relationship between cognitive
with healthy matched control subjects.108 The ambula-
function and magnetic resonance imaging (MRI) anal-
tion ability of those with SB deteriorates over time
ysis of ventricular dilation or parenchymal thickness in
and is negatively correlated with higher neurolog-
young adults shunted for congenital hydrocephalus;
ical lesion level. A comparison of the different sys-
however, the total number of individuals in this study tems used to classify the neurological level in in-
was only ten.100 dividuals with SB is well outlined by Bartonek et
The ability to secure employment also depends al.109 About 92% of individuals with lower level
on the ability to obtain reliable transportation to lesions (S1 and below) ambulate, whereas only
and from work. Higher order processing skills re- 38% of individuals with middle level lesions (L3–
lated to the frontal lobe functioning and visual L5) and 4% of those with higher level lesions (L2 and
organization skills are important in achieving in- above) ambulate.12,110 When MRI is used to define the
dependence with driving.95 The process of organiz- lesion level, similar findings are noted.111 Iliopsas
ing visual data and making immediate judgments strength grade 0 –3 was associated with partial or
has been shown to be more important to successful complete use of a wheelchair, and deterioration in
driving than reaction time.95 However, cognitive mobility was associated with strong iliopsoas/quadri-
testing alone could not discriminate between ceps and weak (grade 0 –3) gluteus medius.112 Addi-
young adults with SB who were drivers or nondriv- tionally, functional independence has been correlated
ers.101 The availability of adapted cars, good paren- with hydrocephalus and the level of lesion; those
tal and community support, and good financial without hydrocephalus or those with hydrocepha-
support have been positively associated with driv- lus and a lesion below L2 were independent for all
ing success.102 Compared with controls, patients functional independence measure domains except
with SB drove less miles per week, had a higher sphincter control.113 Alternatively, those with hy-
number of accidents, had greater difficulties with drocephalus and a lesion above L2 showed in-
route planning, and had greater difficulties access- creased dependence with sphincter control (98%),
ing service stations.103 Vocational rehabilitation locomotion (79%), self-care (54%), support in transfers
programs can be a source of funding to help with (38%), social cognition (29%), and communication
car modifications. Physiatrists should identify (15%).113 Other factors that negatively correlate with
adaptive driving programs that can carefully assess ambulation/mobility include spasticity associated
the needs of this population to enable them to be with tethered cord syndrome (TCS), contractures,
successful. decreased motivation, low-back pain, and major med-
There is very limited data available on the best ical events.12,20,113–116
way to address underemployment. Certainly, voca- Preservation of mobility through rehabilitative
tional rehabilitation programs and education and measures improves physical function and func-
career counselors are important sources of sup- tional independence and, in turn, health-related
port.104 Additional programs may be available quality of life.58,117 Assistive technology and gait
through the Department of Veterans Affairs for aide use for mobility are common in adolescents
children of Vietnam Veterans.105 Neuropsycholog- and young adults with SB.3,12,14,106 Between 36%
ical testing should be used to identify specific cog- and 57%3,12,14,106 use wheelchairs, and increased
nitive impairments, so education strategies can be wheelchair dependence correlates with higher level
used to target those areas. The use of assistive lesions and hydrocephalus.12,14,113 Johnson et al.106 re-
technology has been suggested as a way to enhance ported that 35% of the adolescents and young adults in
higher level cognitive functions, including mem- their study used braces, and 23% used walking aides.
ory and executive function.106 Early assessment Functional walking can be achieved or maintained into
and identification of alternative funding sources for adulthood for subjects with nonfunctional hip flexors
assistive technology equipment, particularly through using a reciprocating gait orthosis.118–120
vocational rehabilitation agencies, are recommended. Gait analysis of those with high-sacral level SB
More research is needed on how identifying and im- showed that through the use of forearm crutches,
proving transportation barriers, especially for those functional ambulation with a gait pattern similar to

December 2008 Rehabilitation and Management of Spina Bifida 1029

 that of unimpaired individuals can be achieved.121 Musculoskeletal System
Yet, even with the use of gait aides, the physical Although there are few reports in the medical
demands of ambulation can be high and may not be literature on musculoskeletal disorders specific to
adequate to complete the activities of daily living. adults with SB, pertinent information can be ob-
Physical strain in ambulatory adults with SB was tained from the spinal cord injury literature that
shown to be 1.4 –2 times higher compared with peers does include a small number of individuals with SB
without disabilities. However, adults with SB using in its findings. Shoulder pain in individuals with
wheelchairs had a lower energy cost for mobility paraplegia has been reported to have a prevalence
compared with controls. This suggests that wheel- of 31–73%,128 –131 whereas wrist pain has been
chairs, if customized appropriately, may make mobil- found in 49 –73% of wheelchair users.131–134 Stud-
ity more efficient.122 There are scant data on the ies have also shown that the prevalence of upper
metabolic consequences of transitioning from being limb pain increases with the duration of spinal cord
ambulatory with SB to a wheelchair user. Although it injury and consequent wheelchair use.131–134 Be-
is possible that wheelchair use may result in more cause wheelchair users rely on their arms for mo-
sedentary behavior, it is also possible that individuals bility, transfers, and most activities of daily living,
may become more active when using a wheelchair if injury to the upper limbs can have significant con-
energy costs of ambulation are high. More research is sequences. Upper limb pain has been linked to a
needed in this area and on how improving design of lower quality of life and increased dependence on
wheelchairs, assistive devices, or orthoses can im- assistants.135,136
prove overall mobility, activity, and participation. It is Interestingly, the prevalence of shoulder pain
also unclear how barriers in access to adequate and in individuals who have used wheelchairs since
appropriate technology may influence these out- childhood has been found to be significantly less
comes. Thorough evaluations by multidisciplinary than that of individuals with adult-onset wheelchair use.
teams experienced in assistive technology prescrip- Sawatzky et al.137 propose that the immature skele-
tion are recommended because seating needs for ton of persons with SB possibly responds better to
those with SB are complex and must account for the repetitive forces of propulsion than those skel-
scoliosis, short limb length, and sensitive areas over etons of individuals who begin using a wheelchair
the primary closure sites. Power features such as tilt, once their skeletal structure is completely devel-
recline, and elevating leg rests should be considered oped. Much of the shoulder pain in wheelchair
because of the many medical conditions for which users has been attributed to injury of soft tissue
these features are prescribed.123 structures. Rotator cuff disorders are most common,
Physiatrists should aim to preserve physical as seen in several arthrographic and MRI surveys of
wheelchair users with shoulder pain.133,138,139 Aside
function of both ambulators and wheelchair users.
from the ergonomics of wheelchair propulsion,
It has been shown that those who participate in a
frequent overhead reaching by wheelchair users
walking program early in life are overall more
can contribute to this overuse injury. Imbalance
independent, are better able to perform transfers,
among the muscles acting on the shoulder joint is
and develop fewer fractures and pressure ulcers
a typical cause of rotator cuff disorders, leading to
later in life than age-matched controls.124 The
unnecessary strain, susceptibility to injury, and
health of wheelchair users can also be preserved
inefficient energy use.140,141
with specific rehabilitation programs, including Preventative measures include joint protection
stretching, resistance training, and aerobic exer- programs, appropriate wheelchair configuration,
cise, that increase biomechanical economy of and teaching of proper wheelchair propulsion pat-
wheelchair propulsion without increasing shoulder terns and transfer techniques. Physiatrists may
or elbow joint stresses.125 Because the kinematics want to consider prescribing pushrim-activated
of manual wheelchair propulsion have been shown power assist wheels for manual wheelchairs to re-
to be similar in adults and children,126 comparable duce the load of propulsion or seat elevators for
training programs can be used in each group. It is power wheelchairs to reduce overhead strain while
particularly important to maximize efficiency of reaching and to enable users to transfer to surfaces
manual wheelchair propulsion because 50% of of varying heights with healthy transfer tech-
forces exerted at the pushrim are not directed niques.142
toward forward motion and may result in repetitive Conservative treatment is the first line of man-
strain injuries.127 The physiatrist should advocate agement, as with individuals without SB; this in-
and encourage daily adaptive exercise in those with cludes relative rest, nonsteroidal antiinflammatory
SB, especially those who use wheelchairs. More drugs, corticosteroid injections, therapeutic mo-
research is needed to elucidate optimal exercise dalities, and exercises performed in physical and
programs for adults with SB. occupational therapy.143 Specifically, an 8-wk scap-

1030 Dicianno et al. Am. J. Phys. Med. Rehabil. ● Vol. 87, No. 12
ula-focused home exercise program was shown to L2, and more than 90% at L1 and cephalad.157
be effective in reducing shoulder pain and improv- Nonoperative treatments using spinal orthoses, ex-
ing function and satisfaction in manual wheelchair ercises, or manipulation may help to improve func-
users with spinal cord injury and SB.144 Favorable tion, but they do not reliably influence the natural
results have also been reported with surgical rota- history of the spinal deformity.157 Indications for
tor cuff repair.145,146 surgical correction include a progressive defor-
Biceps tendinopathy and tendon tears are an- mity, compromise of sitting or standing balance,
other source of shoulder pain. They are often as- skin breakdown, facilitation with nursing care, car-
sociated with supraspinatus tears.147 Edema and diac or pulmonary compromise, and pain unre-
change in tendon width, seen on ultrasound are sponsive to nonoperative interventions.157 The
often the first signs of overuse injury.148 As with combined anterior-posterior approach has been
rotator cuff disease, conservative treatment is in- shown to be more efficacious in older adolescents
dicated. Surgical options are generally indicated if and adults when pelvic obliquities are present.157
symptoms continue after 3 mos of conservative Some research has shown no improvements in
management or if there is biceps instability. These quality-of-life measures after surgery in those with
include tendon debridement, release of constricted SB, which is in contrast to those with other dis-
synovial sheath, tenodesis, and tenotomy.149 abilities.160
Nerve entrapments are a common cause of Charcot arthropathy can lead to significant pain
pain and functional impairment in wheelchair us- and functional decline in individuals with SB. The inci-
ers. Although there are no published reports on dence has been reported to be nearly 1 in 100 cases of
carpal tunnel syndrome (CTS) specific to individ- SB, with a mean age of diagnosis of 15 yrs and 7 mos;
uals with SB, it can be surmised that they are however, this is likely an underestimation.161 The most
similarly affected. Common symptoms include common location for the development of arthropathy is
hand numbness and paresthesias restricted to the the foot and ankle, followed by the hip and knee.161
median nerve distribution, wrist pain worse at Maynard et al.162 demonstrated that foot rigidity, non-
night, and hand weakness or clumsiness.150 Clini- plantigrade foot position, and history of arthrodesis were
cal diagnosis of CTS is supplemented by nerve strong indicators of the eventual development of neuro-
conduction studies and electromyography. These pathic foot changes. Adequate management can usually
electrophysiologic abnormalities may precede clin- be achieved with the use of braces, including AFOs,
ical manifestations, as seen in studies of individuals KAFOs, or HKAFOs161 or patellar tendon-bearing AFOs
with unilateral symptoms, but are found to have for the treatment of Charcot arthropathy of the foot and
bilateral CTS when electrodiagnostic studies are ankle.163
performed.151,152 Initial treatment for CTS includes The prevalence of orthopedic deformities and
rest, activity modification, wrist splinting, corticoste- development of joint contractures is common in
roids via oral route, or intracarpal injection; in more those with SB and can hinder continued mobility.
severe cases, endoscopic or open surgical decom- Those with thoracic level lesions often have equi-
pression is indicated.150,153–155 Weight control may nus foot (55%), those with mid-lumbar lesions
also be important in prevention of CTS because have club foot (87%), and those with sacral lesions
increased body weight correlates with worse me- have a calcaneal foot (34%).110 Knee flexion con-
dian nerve function in wheelchair users.156 There tractures are seen at all levels; however, the ma-
is no published literature available on the preva- jority occur in those with thoracolumbar le-
lence of ulnar neuropathy in those with SB, al- sions.164,165 Positional deformation, spinal reflex
though the physiatrist should also consider this activity, fractures around the knee joint, and weak
diagnosis as part of a thorough evaluation. quadriceps are the main factors associated with
Scoliosis of varying severity is present in flexion contractures at the knee.164 Although sur-
47.7% of persons seen at an adult SB clinic.14 The gical correction of knee flexion contractures is usu-
rates of spinal deformities in SB are related to the ally performed in childhood, recurrence of contrac-
clinical motor level or neurological classification, tures can occur years after surgery and are associated
ambulatory status, and last preserved laminar with lack of ability to stand or walk and obesity.164
arch.157–159 Often, surgical correction is performed Abnormal knee biomechanics can lead to knee
during childhood and adolescence while musculo- symptoms in those with SB. Specifically, it has
skeletal growth and development are still occur- been shown that abnormal stresses, secondary to
ring. New scoliotic curves tend to continue to de- weak hip abductors and calf muscles, placed on the
velop until the age of 15,158 and adults with SB and knee in those with low-lumbar SB can lead to
scoliosis usually have spinal curves that are already medial and anteromedial knee rotary instability
static. Those with sacral level of function have a 5% and eventual degenerative changes.166 Adults
prevalence of spinal deformity with a steady in- with high-sacral SB with abnormal internal knee
crease to 25% at L5, 60% at L4, 70% at L3, 80% at varus moment and an increase in the knee ex-

December 2008 Rehabilitation and Management of Spina Bifida 1031

 tension in stance phase may have early onset of patient wants to resume a therapeutic standing or
degenerative changes if they have not undergone walking program.
tibial osteotomy.167 We found no studies on therapeutic standing,
Hip flexion contractures and hip dislocation bisphosphonates, calcitonin, calcium, or vitamin D
have been problematic in those with SB. Hip flex- for treatment or prevention in SB. Although it has
ion contractures have been correlated with an in- been suggested clinically to add calcium and vita-
creasing lumbar hyperlordosis that is believed to be min D,175 these supplements may be problematic
a way of maintaining center of gravity.168 In regard in the setting of constipation or nephrholithiasis.
to hip dislocation, functional level seems to be Additionally, the presence of gastroesophageal re-
more closely related to neurological level than the flux may prevent bisphosphonate use in some individ-
presence of hip location or dislocation,169 and treat- uals. Oral hydrochlorothiazide is sometimes used to
ment of hip dislocations in ambulatory individuals mitigate effects of urinary calcium loss associated with
with SB offers little additional functional bene- reduced ambulation status175; however, it does not seem
fit.170,171 Alternatively, because unilateral hip in- to affect bone density.182
stability and scoliosis have been shown to be co-
morbid factors,172 it is generally accepted clinical
Neurological and Neurosurgical Issues
practice to perform surgical reduction of unilateral Adults with SB can present with a variety of
hip dislocation in the child or adolescent with SB neurological and neurosurgical issues. Hydro-
to delay or prevent progression of scoliosis or de- cephalus and TCS are the most common, but
velopment of wounds in those who use wheel- others include symptoms from Chiari malforma-
chairs.173 Posteriorlateral transfer of the illiop- tion, syringomyelia, seizures, and chronic head-
soas in those with L3 SB has been shown to have aches.3,10,12,14 –16,20 –22,183 Vigilance in detecting
value in obtaining hip stability and walking abil- signs and symptoms of these issues is imperative
ity.174 Long-term outcome data in large groups given the severity of the outcomes.
Hydrocephalus is a chronic condition that can lead
of adults are not available.
to substantial medical costs.184 It occurs in approxi-
Early-onset osteoporosis occurs in almost 50%
mately 11–90% of those with SB,3,10,16,20 most often
of adults with SB.175 Relevant risk factors for os-
occurring in those with a higher level of lesion.12 In one
teoporosis in this population include female sex,
study of adults with SB and a mean age of 30, approxi-
renal failure, prior ileal diversion surgery, reduced
mately 16% never had a shunt placed, 28% had a shunt
physical activity, use of epilepsy medications or
that was never revised, 18% had shunts that were only
oral corticosteroids, and family history of hip frac-
revised in infancy, and 39% had shunt revisions between
ture.175–178 A study of 21 young adults with a mean
the ages of 2 and 31.98 The highest incidence of shunt
age of 30 showed that common sites are lumbar
revisions tends to occur during the first year of life;
spine, femoral neck, and femoral trochanter.175 however, there is another peak in the number of shunt
Little has been published on the pathophysiology of revisions occurring in the early teenage years that ex-
bone metabolism specifically in SB, but the pattern tends into adulthood.185 Shunt malfunction can occur
of bone density loss in adults seems to follow the for various reasons, including obstruction (distal or
patterns seen in acquired pediatric spinal cord in- proximal), disconnection, fracture, migration, and infec-
jury in which the hip is affected more than the tion.186 Common presenting symptoms include head-
lumbar spine.175,179 Regular screening of bone aches, vision changes, gait disturbances, nausea, vomit-
mineral density is recommended, although a con- ing, and death; however, many times, asymptomatic
sensus on minimum age has not been estab- increases in intracranial pressure occur.185 Hydroceph-
lished.175 Additionally, poor readings may occur alus can also lead to hypothalamic and pituitary dys-
due to obesity, the SB defect, heel deformities, or function that is associated with precocious puberty that
spinal hardware,175 so optimal screening location occurs more commonly in women.187 An insidious and
may be dependent on the individual. mild decline in cognitive or neuropsychological func-
The annual incidence of fracture is 29/1000 in tioning can sometimes be the only presenting symp-
adolescents and 18/1000 in adults with SB.180 Most tom.188 Long-standing overt ventriculomegaly is an en-
fractures involve the femur and tibia. Although tity that refers to a pronounced decrease in intracranial
research on children with SB shows that both compliance secondary to a chronic and slowly progres-
neurological level and actual weight-bearing status sive hydrocephalic state.189 It has been associated with
affect bone density,181 only older age and higher multiple problems, including intermittent headaches,
level have been independently associated with in- subnormal IQ, and psychiatric problems, specifically de-
creased number of fractures in adults with SB.175 pression.189
No data are currently available on the risk of patho- Although there are no standard protocols to fol-
logical fracture with resumption of weight-bearing low hydrocephalus in the adult, radiologic assess-
status after a period of being nonambulatory if a ment should be performed when shunt malfunction

1032 Dicianno et al. Am. J. Phys. Med. Rehabil. ● Vol. 87, No. 12
is suspected. However, about 10% of head CT and when MRI is contraindicated.195 Findings on MRI
MRI studies can show small ventricles in the presence that suggest tethered cord include an elongated cord,
of shunt failure.190,191 Ventricles may be poorly com- posteriorly displaced filum or conus medullaris,
pliant, the shunt may be over- or intermittently thickened filum, or low-lying conus (below L1–L2 or
draining, the shunt may be leaking, or the patient L2–L3), but these findings are not always present on
may have slit ventricle syndrome, a condition in imaging.201 The actual characteristics of the tether-
which the ventricles seem decompressed but revision ing can only be definitely delineated during sur-
may be necessary. Furthermore, small ventricles may gery.201 Somatosensory evoked potential may help in
have preceded malfunction, so it is important for diagnosis and predicting outcomes postsurgery.195
physiatrists to obtain baseline imaging when the Because evidence of a tethered cord is com-
shunt is functioning so that future comparisons can monly seen on imaging studies in asymptomatic
be made in an urgent situation. Neurosurgical eval- individuals,202 surgery should be considered only
uation should be performed regardless of imaging when the clinical presentation supports the diag-
findings if malfunction is suspected because missing nosis of TCS.197 In general, surgical release in TCS
a diagnosis of malfunction can result in death.190 In has led to good outcomes and should be per-
cases in which the ventricles are enlarged, it is like- formed as soon as possible after presenta-
wise important to compare with baseline imaging. tion.195,196,198,199,203,204 Maher et al.198 reported
Not all cases of enlarged ventricles suggest malfunc- postsurgical symptomatic improvement in pain
tion190; however, in cases in which malfunction is (81%), urinary symptoms (53%), and weakness
still suspected, evaluation includes other neurosurgi- (48%). An increasing number of untethering pro-
cal diagnostic modalities, such as shunt X-rays, shunt cedures in those with previous SB repair have been
taps, intracranial pressure monitoring, shunt patency associated with worse outcomes, specifically, poor
studies, inpatient observation, or surgical explora- pain relief and a greater chance of morbidity.198 In
tion.190,192 Endoscopic third ventriculostomy has been addition, those with previous SB repair tend to
another technique to manage hydrocephalus,193 but the have worse overall disability scores and poorer neu-
outcomes are variable, and this technique may make rological outcome than those with a history of
subsequent obstructive symptoms difficult to diag- closed or occult dysraphism.195 Common postsur-
nose.194 gical complications include wound infections, cere-
Tethered cord results from the fixation of the brospinal fluid leakage, pseudomeningocele, and dys-
spinal cord to inelastic structures and typically aesthetic lower limb pain.195,198 Currently, there are
occurs at sites of scar formation from previous no studies evaluating the efficacy of oral neuropathic
surgery.3,10,12,15,195 It is a common finding, and medications such as gabapentin on pain due to TCS.
many individuals remain asymptomatic. It be- The skills of a physiatrist in the detection of func-
comes clinically relevant when individuals de- tional and neurological changes in this population
velop symptoms, in which case it is called are thus crucial for prompt recognition and referral
TCS.195 The most frequent clinical symptoms for neurosurgical intervention.
include motor dysfunction and pain in the back, The most common type of Chiari malforma-
legs, or both.196 The incidence of TCS in those tion in individuals with SB is type II, which is
with SB ranges from approximately 20% to 55% defined radiologically by the herniation of hind-
and is increased in those with lower level lesions brain structures through the foramen magnum.205
or a history of previous tethered cord re- The most common presenting symptoms include
lease.3,10,12,15,195 Specifically, the incidence of TCS bilateral upper limb weakness, sensory changes,
occurring after SB repair has been reported to be dysphagia, headaches, and ataxia.206,207 The preva-
between 3% and 15%, with the incidence of pro- lence of Chiari malformation may be more than
gression increasing with time since SB repair.195,197 75% in adults with SB, and it is likely commonly
Common presenting symptoms include back and associated with syringomyelia.183 It seems only
lower limb pain, weakness, sensory changes, urinary those with radiologic findings of a Chiari mal-
symptoms, foot deformities, and scoliosis.195,198,199 formation develop symptoms, but absence of
Those with suspected TCS are commonly advised to symptoms is not predictive of a normal MRI.183
avoid flexion, twisting, and heavy lifting, but there are Typically, surgical decompression of the posterior
no published data to delineate spinal precautions in fossa and upper cervical spine is performed when
adults. symptoms associated with radiologic findings are
Common findings on physical examination in- present. Early intervention improves the chance of
clude sensory loss, hyperreflexia, spasticity, clonus, neurological and functional recovery.206
and Babinski’s sign, but they may not appear until Hydrosyringomyelia technically refers to a col-
dysfunction is advanced.200 MRI has become the lection of cerebrospinal fluid in the central spinal
“gold standard” for detecting tethered cord, but CT cord, and syringomyelia refers to collections that
myelogram has also been used, especially in cases are paracentral; however, syringomyelia is often

December 2008 Rehabilitation and Management of Spina Bifida 1033

 used as the collective term, and we will use it here Renal/Urological
to refer to both findings. The prevalence of syrin- Bladder disturbances commonly affect be-
gomyelia in the adult population with SB is not tween 24 and 94% of children and adults with
available. Cervical syringomyelia has been associ- SB.12,16,20,21,211,212 Those with SB aperta, hydro-
ated with Chiari malformations as mentioned cephalus, and a lesion level of L5 or above are more
above,183,206 but its incidence occurring at other likely to have urinary and/or fecal incontinence211;
levels of the spinal cord is unclear. Terminal syrin- however, it has been proposed that cervical and
gomyelia, cystic dilation of the lower third of the upper thoracic SB are associated with a lower risk
spinal cord, is commonly associated with occult of voiding disorders.213 Urinary sepsis is a common
spinal dysraphism and a tethered spinal cord.208 reason for hospital admissions,214 and renal failure
Large fluid collections are usually symptomatic is often cited as the most common cause of death
and are typically associated with motor impair- in adults with SB.9 Those undergoing early neuro-
ments, sensory impairments, scoliosis, and bowel surgical treatment (before age 1.5 yrs) are more
and bladder dysfunction.208 MRI is typically the pre- likely to have normal bladder function in the long-
ferred imaging modality, and surgical intervention term,215 and the presence of perineal sensation in
usually consists of suboccipital decompression or infancy is associated with the long-term outcomes
shunting of the syrinx into the subarachnoid space, of improved survival, decreased renal complica-
the peritoneum, or the pleural cavity.206,208,209 Sur- tions, and the likelihood of continence.216
gery should be considered when a syrinx is clinically SB can affect the bladder in a number of ways.
or radiographically significant.208 No literature is cur- Combined degeneration and regeneration in the
rently available on the use of electrophysiological intrinsic nerves of the detrusor have been noted in
studies in the evaluation of syringomyelia. upper motor neuron neurogenic bladder dysfunc-
Chronic headaches have been shown to be a tion,217 and retention is the most common bladder
frequent problem in the adult with SB. The etiol- pattern.218 Commonly reported symptoms include
ogy of headaches in this population is diverse and urgency, frequency, incontinence, hesitancy, slowed
has been reported as follows: unknown etiology stream, double voiding, and inability to void after
(35%), shunt blockage (25%), decompensation of feeling of urge.218 Retention of less than 100 –200 ml
previously arrested hydrocephalus (10%), Chiari is considered acceptable.
malformation (8%), scar neuralgia (6%), tension Urodynamic testing is considered the gold standard
headache (4%), migraine (4%), endoscopic third for diagnosing neurogenic bladder. A wide spectrum of
ventriculostomy failure (4%), shunt over drainage urodynamic abnormalities have been observed, in-
(2%), and choroid plexus coagulation failure cluding upper and lower motor neuron types of blad-
(2%).207 Evaluation of headaches in this population der and urethral dysfunction.219,220 Those with a
should first consist of ruling out life-threatening overactive sphincter have the best chance of be-
etiologies, including neuro-imaging and intracra- coming continent.220
nial pressure monitoring if indicated by clinical Many treatments for urinary incontinence are
history and findings. If a definitive etiology is not available for individuals with SB.221 Achievement
identified, then specialized headache pain manage- of urinary continence is associated with a better
ment should be pursued. Ten percent of hydroce- self-concept.222 Anticholinergic agents are the main-
phalic adults with SB have been noted to require stay of treatment.218,223,224 Oxybutynin and tolterod-
specialist pain management for control of chronic ine are first generation anticholinergic medica-
idiopathic headaches.207 tions commonly used. Side effects such as dry
The incidence of epilepsy and seizures ranges mouth, constipation, and blurred vision may limit
from 3% from 23% in the adult SB popula- the amount of drug one can tolerate.224 Newer
tion.3,12,14,21 The incidence increases with higher antichiolinergic medications, including solifena-
level lesions,12 and the etiology is most likely mul- cin, darifenacin, and trospium,224 are supposedly
tifactorial.210 Adequate control of seizures is inte- more selective at blocking cholinergic receptors on
gral to reducing morbidity and maximizing qual- the bladder than in the periphery, but long-term
ity of life. outcome data are lacking. Imipramine is a tricyclic
Early-onset osteoarthritis of the spine may also antidepressant that has been reported to limit blad-
occur. Lumbar canal stenosis could be a potential der contractions and improve storage.224 Like all
cause of neurological deterioration. More research tricyclic antidepressants, imipramine can also
is needed on treatment of spine disorders, partic- cause undesirable side effects such as dry mouth,
ularly in the area of interventional spine proce- constipation, and dizziness. For cases of refractory
dures, as the unique approaches used and their nocturia or enuresis, desmopressin acetate is used
efficacies have not been documented in individuals to limit urine production and is available in tablet
with SB. form or as a nasal spray.224 Doxazosin mesylate,

1034 Dicianno et al. Am. J. Phys. Med. Rehabil. ● Vol. 87, No. 12
tamsalosin hydrochloride, and terazosin hydro- appliance can be placed over the opening to collect
chloride are examples of alpha-1-adrenergic antag- urine or the stoma, which can be created in the
onists, and they can be used in both men and navel, can be catheterized intermittenly. In few
women due to spasticity of the internal sphincter, instances, enlargement of the bladder via augmen-
pelvic floor, or bladder neck. Side effects of these tation cystoplasty or autoaugmentation may be in-
medications include dizziness or headache. Be- dicated with very small bladder capacity and asso-
thanechol stimulates cholinergic receptors and ciated urinary incontinence.240 –243 During this
helps the bladder contract more forcefully so that major surgery, the bladder is expanded using in-
urine can be expelled. testinal tissue or engineered bladder tissue, which
When symptoms of retention are not resolved is a relatively recent advancement.244 Electrolytes
adequately with behavioral techniques and/or med- need to be monitored periodically in those who
ications, catheterization should be considered. have undergone bladder augmentation, specifically
Many individuals report that self-catheterization ileal augmentation, as serum bicarbonate and chlo-
improves quality of life and self-esteem.225,226 Al- ride levels can be abnormal.245 The electrolyte ab-
though women tend to accept the concept more normalities seem to normalize during long-term
willingly than men, the procedure is more difficult follow-up.246
for women. Those who have sphincter hypereflexia, A more recent innovation is the injection of
have detrusor hypo- or arelfexia, and regularly per- botulinum toxin into the urethra or blad-
form bladder emptying using clean intermittent der.218,224,247,248 Although injection effects are not
catheterization have been shown to have the best permanent, they can provide improvement without
success in achieving social continence.227 Feasibil- major surgery.247,248 In those with overactive bladder,
ity and practicality can be influenced by a number injections into the bladder can help to relax the muscle
of other factors, including limited manual dexter- and thereby, decrease frequency, urgency, and or incon-
ity, lower limb spasticity, cognitive impairment, tinence while increasing volume within the bladder.247
availability of assistance, and accessibility of public Repeat injections may be necessary after 6–12 mos.247
restroom. Catheterization is recommended to be Intravesical oxybutynin is another option for treatment
done at 3– 6 times per day.225 There are relatively of neurogenic bladder.218,224,249 Its efficacy has been
few complications associated with clean intermittent reported to be 66–87% depending on the dosage giv-
catheterization, the most common being macro- en.250 Immediate posttreatment cystometrogram was
scopic hematuria and infections.228 –230 We found no predictive of the overall response to intravesical oxybu-
evidence that there is an association between the tynin treatment on follow-up studies.251 Earlier studies
number of urinary tract infections and either the using transurethral electrical stimulation for the reha-
type of catheter used (sterile vs. clean reused) or bilitation of the neurogenic bladder showed mixed to
prophylactic antibiotics.229,231 Asymptomatic bac- poor results.252,253
teriuria in those using clean intermittent catheter- Without adequate urological management, the
ization does not require antibiotic therapy unless upper urinary tract can be adversely affected. Br-
vesicoureteral reflux is present.232 However, the uschini et al.254 demonstrated that 26% who were
main reason for emergency room visits in this urologically untreated had evidence of kidney dam-
population is urinary tract infection.233 A long- age. Specifically, a detrusor leak point pressure
term indwelling catheter should only be used as a more than 40 cm H2O and a decrease in functional
short-term method to manage bladder emptying in bladder capacity to less than or equal to 33% was
individuals whose bladder cannot be fully emptied associated with upper urinary tract damage.254
and for whom intermittent catheterization is not an Early and appropriate initiation of urological man-
option. A suprapubic catheter can be used to manage agement will help to safeguard renal function in
bladder activity in these individuals. those with SB and may preserve renal function for
Stress incontinence can often be managed by a lifetime.223,255,256 Screening for renal disease
injection of collagen or other biocompatible mate- should be performed routinely in SB.257 Typically,
rial into the urethra.218,234 Although periurethral ultrasonography is used, but magnetic resonance
collagen injections showed initial promise, the urography is being investigated and shows prom-
long-term results have been poor and are likely ise, especially when imaging is difficult due to body
secondary to degradation of the collagen.235 Addi- habitus.258,259 Persun et al.260 showed that none of
tionally, artificial sphincters have been used, but the adults with SB in their study with a normal
their limited long-term survival has brought into renal ultrasound and normal serum creatinine
question their long-term use.236 Surgical sling and (⬍1.5 mg/dl) had evidence of increased bladder
bladder neck wrap operations are another option pressure on cystometry. They recommend yearly
for urologic dysfunction in those with SB.237,238 renal ultrasound and serum creatinine for screen-
Several types of urostomies exist: ileal conduit, ing and urologic consultation and urodynamics
colonic conduit, and appendicovesicostomy.239 An only if abnormalities were noted.260 However, se-

December 2008 Rehabilitation and Management of Spina Bifida 1035

 rum creatinine alone is an unreliable predictor of Fewer than 25% of men with SB at T11-L2 can
renal function in those with SB. Specifically, Quan achieve either psychogenic erections, which are
et al.261 showed that once the serum creatinine is mediated through the corticospinal tracts and pu-
greater than 0.5 mg/dl, glomerular filtration rate dendal nerves or reflexogenic erections, which are
determined from a clearance study is the only mediated through spinal cord reflex arcs.187 The
reliable method to assess renal function in individ- presence of an anocutaneous reflex has been asso-
uals with SB.261 Renal transplant has been used ciated with ability to achieve erection even at these
successfully in individuals with SB and should be lesion levels.187 Almost 75% of men with SB at or
considered as a possible treatment for end-stage below L3 have erections, and most in this group
renal disease.262–265 have some penile sensation.187 However, many
Various other renal and urologic complica- men are sexually active regardless of whether
tions have also been identified in adolescents and they have sensation or are able to achieve erec-
adults with SB. Bladder cancer incidence is similar tion.280 Overall, most men with SB begin sexual
to the general population but can present at a activity later than unimpaired peers281 and have
young age, be more invasive, and be associated lower sexual satisfaction than women with SB.282
with poor survival.266 Associated risk factors for The only randomized, controlled trials we en-
bladder cancer have not been described fully; how- countered in this review were two studies reporting
ever, the urothelium is frequently abnormal in significant improvements in erectile dysfunction in
young adults with SB who are performing inter- men with SB with the use of silendafil.283,284 Other
mittent catheterization.267 treatments available for erectile dysfunction are
Bladder and renal calculi are associated with penile prostheses, vacuum pumps used with con-
neurogenic bladder in the young adult with striction rings, prostaglandin E1, and papaverine
SB.268,269 The exact etiology of stone formation is injections with or without phentolamine.187 There
unclear, but history of urinary diversion, bladder has been one report of ilioinguinal nerve bypass to
augmentation, and urinary tract infection all cor- restore sensation to the dorsal penis.285
related significantly with urolithiasis.269 Stone Those with sacral lesions are usually able to
analysis in one study demonstrated calculi com- ejaculate,279 but this is uncommon for higher le-
posed of struvite (39%), calcium phosphate (35%), sions. Transrectal electrical stimulation and vibra-
uric acid (13%), and calcium oxalate (13%).269 tor stimulation can be used in some cases.187 Male
Advances in urologic treatment of neurogenic blad- infertility may be due to erectile dysfunction, ret-
der have reduced infections, and metabolic stones are rograde ejaculation, primary testicular failure from
now more commonly identified.268 Compared with cryptorchidism, or medications such as chronic
extracorporeal shock wave lithotripsy, endoscopic antibiotics or tricyclic antidepressants.187 Sexual
procedures are the preferred treatment of renal cal- development, sexual function, prostatic growth, or
culi in those with a urinary diversions.270 prostatic morphology have been shown not to be
altered by placement of an artificial urinary sphinc-
Sexuality ter around the bladder neck.286 Reproductive po-
Sexual education is an important part of reha- tential is favored by lower and less severe le-
bilitation care,271,272 but very few individuals get sions.287 Men with lesions above T10 are at higher
sexual education from physicians.273 Impairments risk for azoospermia.288
of cognition and manual dexterity, inadequate sex- There is an increased risk of neural tube de-
ual education, overprotective parenting, and lack of fects in children of fathers with SB, but there has
information from healthcare providers can impede been scant research on the role paternal folate may
healthy sexual development.187 Furthermore, lack play in prevention.288 Nutritional counseling
of social relationships and poor self-perceptions of should be part of routine care for those with SB.289
body image can delay emotional maturation. Over Folic acid supplementation has been shown to be
95% of individuals with SB report they need more as effective in increasing blood folate levels in in-
information about sexuality and fertility274; yet, dividuals with SB when compared with controls with
most are sexually active or intimate with a partner other disabilities.290 In women of child-bearing age
despite not having adequate information.275,276 with SB, current recommendations are for 4 mg of
One study showed those with hydrocephalus may folate supplementation daily, which is 10 times the
be less active than those without hydrocephalus.277 typical daily dose recommended.291
Another survey showed that almost 25% of adoles- More than 90% of women with SB at or below
cents with SB knew nothing about conception or L3 have sexual sensation and more than 40% are
contraception.278 Moreover, those with inconti- able to have orgasms.279 Lubricants are recom-
nence are less likely to be sexually active.273 In mended for those who experience dryness.187
both men and women, continence is associated Women are usually fertile. Pregnancy, however,
with being more sexually active.279 may be complicated by increased neurogenic blad-

1036 Dicianno et al. Am. J. Phys. Med. Rehabil. ● Vol. 87, No. 12
der symptoms, urinary tract infections, and by pel- traction in response to rectal distension.302 Bowel dys-
vic deformities that may make delivery difficult.187 function can be a cause of abdominal symptoms in
Genetic counseling is also necessary given that individuals with SB, but other etiologies need to also be
both men and women with SB have an increased considered. Worley et al.303 identified neurogenic blad-
risk of having a child with SB.187 Although obvious der (41%), neurogenic bowel (14%), ventricular perito-
for some clinicians, others may need reminding neal shunt complications (18%), and complications
that not all individuals with SB are heterosexual.187 from previous surgeries (9%) as the most frequent
Thus, counseling on sexual matters should be tai- causes of acute abdominal symptoms in children and
lored for sexual orientation. young adults with SB.
Important considerations when discussing The goal of any bowel program in an individual
contraceptive measures are the manual dexterity with SB should be to create a scheduled and pre-
required to use the contraceptive device, risk of dictable bowel movement that will adequately evac-
thromoboembolism with oral contraceptives, and uate stool from the colon. Better bowel continence
the importance of using latex-free condoms.187 can be achieved through emphasizing patient/fam-
There are no studies that suggest a preferred ily education and a regular, consistently timed,
method of contraception. Education should also be reflex-triggered bowel evacuation.304 Additionally,
given on masturbation, hygiene during menses, sex- Leibold et al.296 formulated an algorithm for step-
ually transmitted diseases, and sexual abuse. Both men wise decision-making to help individuals with SB
and women may experience incontinence during or- and their healthcare providers construct a success-
gasm and should be counseled on emptying the bladder ful continence program. The protocol contains 13
before sex. However, painful bladder spasms can also assessment points, including stool consistency, fre-
occur with an empty bladder.187 quency and amount; mobility; level of paraplegia;
Most published research on pregnancy in SB is diet; medication; anal/rectal canal tone; prior pro-
descriptive or case study in nature, and much of grams attempted; family routines; age; accessibility;
this literature is up to three decades old.292 Al- and learning issues.296 The best bowel program is the
though the literature supports low complication one that maintains continence and best fits the indi-
rates and positive outcomes with pregnancy,293 de- vidual’s lifestyle.305 This is typically achieved through
creased mobility and increased rates of pressure a combination of treatments.296,306 Bowel programs
ulcer development have been noted.293 Although will likely need to be adjusted to accommodate the
anatomy is an important consideration during pro- changing needs of the individual as he or she ages
cedures, no prospective studies have evaluated and the social situation changes.306 Key elements
placement of epidural anesthesia catheters or out- for monitoring a continence program include the
comes of cesarean section. In general, vaginal de- degree of constipation and its etiology; changing
livery is preferred, and caesarean section should be age; family availability for assistance; wheelchair
performed for obstetric reasons only.294 The pres- accessibility of the toilet; and ability to transfer to
ence of a shunt and altered anatomy due to uro- and from the toilet.296
logical surgeries should be noted when cesarean Diet modification, including the addition of
sections are considered.294 We did not find any high fiber, green tea, and coffee to increase stool
studies that focused on the adult with SB as a bulk and regulate bowel motility has been de-
parent. scribed.307 Patients may benefit from limiting or
avoiding the following foods: citrus fruit, fruit
juice, passion fruit, pineapple, corn, baked beans,
Bowel Management chocolate products, and nuts. To soften stools, a
The psychosocial effects of neurogenic bowel diet rich in fiber, fluid, and fat, but moderate in
can be profound. Incontinence alone is likely not sugar may prove beneficial. Manual evacuation and
the sole determinant of health-related quality of digital stimulation are also commonly used, with
life295; however, it acts as a social stigma for chil- or without the concurrent intake of oral medica-
dren and a barrier for adults seeking employ- tion.211 Medications used in bowel management of
ment.296 Sixty-six to 83% of those with SB perceive children and adults with SB include stool soften-
fecal incontinence as a problem.211,297 Most individ- ers, laxatives and bulk formers. Enemas and sup-
uals suffer from constipation, fecal incontinence, or positories can be used to facilitate bowel evacua-
both.298,299 Voluntary retention of stool is often com- tion, and large-volume retrograde colonic enemas
promised due to a highly compliant rectum and weak can be used every 1 or 2 days to reduce soiling302,308,309;
external anal sphincter.300 Impaired reflex arcs between however, the disadvantage of cleansing only the left
the colorectum and sacral spinal cord result in reduced side of the colon is noted with this technique.302
emptying of the rectosigmoid and descending colon.301 Biofeedback has been somewhat successful in those
Constipation may also occur because of increased transit 5–16 yrs old with SB,310 but other studies have shown
time through the colon and the absence of reflex con- mixed results.311

December 2008 Rehabilitation and Management of Spina Bifida 1037

 Surgical procedures are often used for fecal creasingly important. Renal failure is still cited as
incontinence that is refractory to conservative the most common cause of death in adults with SB,
treatments. A continent appendicostomy (Malone but cardiac disease is becoming more common and
procedure) connects the appendix to the umbili- is cited in one study as the second leading cause of
cus, and the new orifice can be used to administer death.9 Future studies should focus on elucidating
an antegrade colonic enema.312–315 Reports in the the prevalence, determining screen protocols, and
literature describe positive clinical experiences with this determining the best management of cardiac and
procedure, including reliable colonic emptying, de- endocrine disease in adolescents and adults with SB.
creased fecal soiling, high satisfaction, and increased
independence with regard to bowel function.302,312–316 Skin
Other options include cecostomy or ileal conduit.317,318 The management of wounds is complex and
These can be performed surgically, endoscopically, or beyond the scope of this article; furthermore, most
radiologically to provide some of the same benefits as published literature on this topic is not specific to
an appendicostomy while sparing the appendix for SB. The cost of skin care in the SB population is
urinary tract reconstruction.317,319 A high degree of very large. In an older study by Harris and
satisfaction has been reported with this proce- Banta,331 2 million dollars and the equivalent of 17
dure.317,319 Complications from either procedure yrs in hospital days were needed to treat skin dis-
include the formation of granulation tissue, steno- orders in 75 patients with SB during a 13-yr period.
sis of the stoma, dislodging of the tube, and leakage Additionally, wound infections after surgical pro-
of the irrigation solution.302,314 –317,320 cedures commonly occur in individuals with SB,
There were no specific protocols or recom- and they are often polymicrobial and caused by
mendations specific to adult bowel management more gram-negative organisms than what is typi-
identified in our search. Overall, the above-men- cal.332 Another skin complication includes burns
tioned treatments are adapted to each individual. that can occur to the insensate limb, especially
Further research is needed to determine which when modalities are used in therapy.333
bowel programs or treatments are most likely to be
effective in adults with SB. Thromboembolism
The literature is sparse on deep venous throm-
Cardiac/Endocrinology boembolism (DVT) in those with SB. We only iden-
The prevalence of metabolic syndrome is more tified one retrospective review334 and one case se-
than 30% in adolescents with SB.321 Individuals ries in our search.335 Individuals with SB have
with SB have a significantly higher percent body fat multiple inherent risks for the development of
than individuals with no impairments and normal DVTs, including venous stasis or lymphedema, im-
weights, but this percentage is similar to the gen- mobility, paraparesis, obesity, and significant sur-
eral overweight population.322 Excess adipose tis- gical history.334 Levey et al.334 report that 2% of
sue increases with age while height decreases with children and adults in their study developed DVTs.
age when compared with reference data.323–325 Be- The risk of DVT increased in the mid-teen years
ing overweight seems to affect quality of life of and was associated with higher motor level le-
those without impairments more than it does for sions.334 Studies should be performed to further
individuals with SB.326 Aerobic capacity with lower elucidate risk factors and preventative strategies
peak VO2, total lean mass, resting energy expendi- for DVTs in this population. DVT prophylaxis
ture, and upper limb strength are significantly re- should be highly considered for young adults with
duced in individuals with SB.322,327,328 Rehabilita- SB who are nonambulatory and are undergoing
tion protocols should target upper limb strength significant orthopedic, neurosurgical, or abdomi-
with resistance training programs combined with nal surgeries.334 Additionally, there is some evi-
aerobic activities such as swimming and ergometry dence that suggests lymphatic dysfunction may be
hand cycling. Performance may be limited by pe- present in SB.336 Lymphedema should be distin-
ripheral arterial pooling of blood and subsequent guished from lipedema, which is lower limb en-
reductions in cardiac preload, but low-intensity, largement due to fatty deposition.
short duration training with arm ergometry can
increase peak oxygen intake and cardiac stroke Latex Allergy
volume.329 Additionally, Widman et al.330 showed The prevalence of latex sensitization has been
that adolescents with SB who used an upper limb reported to be between 40 and 48% in two recent
hand cycle integrated with a video game at least 3 studies,337,338 with only about 15% exhibiting clin-
times per week for 16 wks were able to improve ical allergic reactions to latex.338 There seems to be
oxygen uptake and maximum work capability. As a propensity for those with SB to develop latex
individuals with SB continue to live longer, main- allergies compared with others who undergo sim-
tenance of good cardiac health will become in- ilar types of procedures as children.337,339,340 When

1038 Dicianno et al. Am. J. Phys. Med. Rehabil. ● Vol. 87, No. 12
compared with healthcare workers, those with SB opment.349 Routine screening for visual impair-
had a markedly different and more severe immu- ments, especially strabismus, and the associated com-
noglobulin E response to proteins and allergens in plications should be performed on all individuals with
rubber latex.341 There has been some debate re- SB to ensure that they maintain their vision into and
garding the contribution of associated atopy, asso- throughout adulthood. Although there are not stan-
ciated family history of allergies, age, presence of dard protocols for screening, at a minimum annual
shunt, and the number of surgical operations to ophthalmologic examination should be considered.
the development of latex allergy.339,342–344 Nigge-
mann et al.345 showed an increased number of LIMITATIONS AND FUTURE DIRECTIONS
operations and the presence of a shunt were asso- One limitation of this article is the singular
ciated with an increased risk of developing sensiti- use of PubMed as a database. Certainly, there is a
zation and allergy to latex. However, recent studies wealth of data on how neuropsychiatric challenges
have not shown an association between the num- may complicate the medical and rehabilitation care
ber of surgical operations and the presence of a of adults. Future work is needed on condensing
shunt with the development of latex sensitiza- and reviewing this literature. Additionally, this
tion.339,342,343 It has been theorized that sensitiza- study includes references from older studies that
tion may occur after only the first operation.340 may be less relevant for a contemporary cohort of
Nevertheless, latex allergies are very common in patients. However, we have taken care to report
those with SB, and a latex free environment should only relevant information from older articles. We
be maintained at all times when treating these feel the inclusion of 20 yrs of research is justified
individuals regardless if they have known latex given that many landmark articles were published
sensitization or allergy. Prophylaxis via environ- in this timeframe. Also, since the reader now has
mental changes whereby a latex-free environment access to the last 20 yrs of literature in electronic
is observed could lead to a reduction in latex sen- format in PubMed, we wished to consolidate what
sitization.346 Rubber stoppers within syringes used he or she may encounter in online searches. A final
for joint or trigger point injections can contain limitation is that we did not include studies that
latex, and cross-contamination could theoretically focused only on individuals younger than 19 yrs.
occur when using multiple use vials of medica- Although some of this literature is potentially rel-
tions, but there have been no studies on this topic. evant to the adult population, review of the pedi-
Patients with confirmed latex allergy may also ben- atric literature was beyond the scope of this article,
efit from nutrition education regarding the poten- but should certainly be a topic of future literature
tial for immunologic cross reactivity between latex review.
proteins and various proteins in foods. Some ex-
amples include avocadoes, bananas, kiwis, and CONCLUSION
chestnuts. As medical treatment continues to improve for
individuals with SB, they will continue to live
Ophthalmology longer and will encounter a wide range of medical,
Visual disturbances often occur in individuals rehabilitation, and psychosocial complications. Fu-
with physical disabilities, including individuals ture research needs to focus on determining the
with SB.347 Papilledema can be an indication of incidence, risk factors, preventative measures, di-
hydrocephalus, but papilledema is not always agnostic options, management options, and treat-
present with shunt failure.191,348 Vision can some- ment options of these complications. Optimal
times improve after shunt revision; however, there management of these complications would allow
are no outcomes studies specific to adults. Other adult individuals with SB to maximize their func-
ophthalmologic disorders are also relatively com- tion and quality of life.
mon in patients with SB, including strabismus
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