The Journal of the Arkansas Medical Society Med Journal Jan 2020 | Page 12

EDITORIAL PANEL: Chad T. Rodgers, MD, FAAP | Elena M. Davis, MD, MPH | Shannon Edwards, MD | William L. Mason, MD | Michael Moody, MD | J. Gary Wheeler, MD, MPS
New Guidelines Direct Care
for Children with Spina Bifida
LAURA J. HOBART-PORTER, DO, FAAPMR
S
pina bifida is a condition affect-
ing 3,000 children every year. 1
It results from the failure of the
neural tube to close during
gestation. Caused by a heteroge-
neous combination of environmental
and genetic factors, spina bifida has
myriad effects on the developing
child, ranging from limb paralysis and
orthopaedic deformities to neuro-
psychological differences. The Spina
Bifida Association recently published
a comprehensive guideline on the
care of people with spina bifida, from
birth through adulthood. 2
Children with spina bifida often
require multiple specialists includ-
ing rehabilitation, neurosurgery,
orthopaedics, urology, plastic
surgery, neurology, pulmonology,
nephrology, endocrinology, gyne-
cology, immunology, physical and
occupational therapists, speech and
language pathologists, dietician,
orthotist, neuropsychologist and
social worker. Coordination of spe-
cialists requires a dedicated interdis-
ciplinary program to assist parents
in navigating the medical and social
aspects of rearing a child with spina
bifida. These services are best deliv-
ered in a multidisciplinary setting
160 • The Journal of the Arkansas Medical Society
that allows for care coordination and
shared decision making. 3,4 The Spinal
Cord Disorders Program at Arkansas
Children’s Hospital provides collec-
tive expertise to coordinate manage-
ment of this complex condition.
In a typically developing embryo,
the neural tube closes at approxi-
mately day 27. Neural tube defects
(NTDs) encompass all forms of
impaired neural tube closure includ-
ing spina bifida (lipo and myelomenin-
gocele), encephalocele, sacral agen-
esis, anencephaly and iniencephaly.
Closure failure can occur at any point
along the spine, most commonly in
the lumbosacral region. Placental fluid
on the exposed nerves is neurotoxic.
The open neural tube alters pressure
gradients within the developing cen-
tral nervous system, which may have
long-term neuromuscular, intracranial
and cognitive implications. 5
Etiologies of NTDs include folate
deficiency, genetic mutation or other
maternal factors (hyperthermia,
exposure to solvents or valproic acid,
obesity or diabetes). Folic acid is the
most potent measure to prevent
neurulation. 1 All women of child-
bearing potential should take 0.4
mg of folic acid daily to help prevent
NTD. Women who have had a previ-
ous NTD pregnancy, maternal NTD or
other risk factors should take 4 mg.
of folic acid daily. 4
Prenatal consultation should
occur shortly after NTD identifica-
tion (18–24 weeks gestation), giving
parents an opportunity to explore all
options, including prenatal repairs
(not available in Arkansas). Intra-
uterine repairs are typically done no
later than 25–26 weeks gestation.
Though intrauterine repair does not
typically help with urologic function
or motor-sensory outcome, it may
decrease the rate of shunt depen-
dence from approximately 80% (in
post-natally repaired children) to
40%. There is a small risk of fetal
loss and risk of uterine rupture with
future pregnancies. 6
Neonatal intensive care unit
(NICU) stay after delivery can vary
from days to months. While in NICU,
the child should be seen by a team
of specialists. Plastic surgery should
be involved for complex defect
closures. Head and renal ultrasounds
are recommended; hips and spine
may also be imaged.
It is imperative that NTD infants
have therapy arranged upon NICU
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