Spina Bifida

Overview and management

Introduction
• the neural tube is the embryonic precursor to the central nervous system, which
is made up of the brain and spinal cord.
• Normally, NEURAL TUBE develops at 3rd to 4th week of gestation
• Neural tube defects (NTDs) are a group of congenital malformations of the central
nervous system that occur when the neural tube fails to close properly during
embryogenesis.
• Causes :
• The causes of NTDs are complex, with both environmental and genetic contributions.
• Maternal nutrition
• Folic acid deficiency
• Maternal diabetes and/or obesity
• Chromosomal abnormalities
Introduction
• “Split Spine” caused by incomplete closure of the neural
tube, usually in the lumbar or sacral region
• Incidence and epidemiology : Worldwide incidence is
2–8 per 10,000 live births
• spina bifida at 1.9 per 1000 births in india
Definition

• Spina bifida is a congenital malformation in which the spinal column is

split (bifid) as a result of failed closure of the embryonic neural tube,
during the fourth week post fertilization.
Classification

• Spina bifida is further divided depending on location and

extent of opening :
1. spina bifida occulta.
2. spina bifida cystica
 meningocele
 myelomenigocele
3. Spina bifida ventralis
• 1. SPINA BIFIDA OCCULTA: In this type of neural tube defect, the meninges do not
herniate through the opening in the spinal canal.
• Most patients are asymptomatic and lack neurologic signs, and the condition is usually of
no consequence
• The skin may have a lipoma (small benign fatty tumor), some
discoloration (birthmark), or a small tuft of hair overlying the
spinal defect
• Most patients with spina bifida occulta do not know they have it
• There may be tethering of the spinal cord
• Tethered spinal cord :
• Often occurs later in life
• Caused by limitations of movement of the spinal cord within the spinal
column
• Patients often have low back pain, weakness in the legs, and/or
incontinence depending on the site of tethering
2. SPINA BIFIDA CYSTICA: includes
• MENINGOCELE which is characterized by herniation of the meninges
• The bony vertebra is open, part of the meninges is
protruding out of the spinal canal
• Since the spinal cord is not protruding, there is often
normal function & Some cases of tethering have been
reported
• MYELOMENINGOCELE (MMC), which involves herniation of the meninges as well
as the spinal cord through the opening.
• As does hindbrain herniation (chiari ii malformation) and associated hydrocephalus
which often requires shunting.
• Orthopedic abnormalities including talipes (club foot), contractures, hip dislocation,
scoliosis and kyphosis are frequently observed.
• Urinary and fecal incontinence occur frequently,
• Spina bifida ventralis – anterior opening
• Much less common than other forms of spina bifida
• Meningeal sac will protrude into the retroperitoneal space
and impinge on retroperitoneal organs such as the
duodenum, ascending/descending colon, kidneys, adrenal
glands, pancreas, aorta, and inferior vena cava
CLINICAL FEATURES
• Primary clinical pictures
1-Abnormal nerve conduction, resulting in:
• Somatosensory losses
• Motor paralysis, including loss of bowel and bladder control (all are below
level of lesion)
• Changes in muscle tone

• Motor and sensory neurological deficit below the level of the lesion results in
lower limb weakness or paralysis that hampers or prevents walking, and lack of
sensation that enhances the risk of pressure sores.
• 2-An enlarged head caused by: 1- hydrocephalus (“water on the
brain”) 11-Arnolds Chairi malformation
• Hydrocephalus VP Shunt done in 85-90% of MMC (1/3 will require
revision at some time)
 Most commonly due to obstruction of Cerebrospinal Fluid (CSF) Flow
• Arnold-Chiari type II Malformation : Medulla, Pons, 4th ventricle +/-
cerebellar vermis herniated into the cervical spinal canal
 Incidence: 80-90% of those with MMC
• Should be managed and treated as an emergency by (surgical
decomperssion operation)
3. Urinary and Bowel symptoms Urinary symptoms:
 In lumbosacral spina bifida cystica, few children attain
urinary incontinence
They require urodynamic testing, including
cystometrography, uroflowmetry, and EMG of the urinary
sphincter
• Bowel symptoms
A. Constipation
B. Incontinence
Management techniques
-enemas -suppositories
-habit training - digital stimulation
-biofeedback -
appendicostomy(ACE)
4-Musculoskeletal deformities (scoliosis)
5-Joint and extremity deformities - joint contractures,
club foot, hip subluxations, diminished growth of non-weight
bearing limbs
DIAGNOSIS

Biochemical diagnosis and screening

• Elevated concentration of alphafetoprotein (AFP) in amniotic fluid samples from pregnancies with
anencephaly or MMC.
 Sonographic diagnosis

• The fetal spine can be examined by ultrasonography in the sagittal, axial and coronal planes from late first
trimester onwards, providing the principal and most accurate mode of prenatal diagnosis.
• Detailed systematic examination is required in all three planes along the entire length of the spine, from
cervical to sacral.
• The ‘lemon’ and ‘banana’ signs were described. These cranial signs have been a significant aid to
prenatal diagnosis, since the head is examined routinely in all fetuses in the second trimester.
MEDICAL MANAGEMENT
• Management and supervision of a child and family with a
myelomeningocele require a multidisciplinary team approach, including a
nurse specializing in the care of children with multiple handicaps, a
pediatric neurosurgeon, urologist, and orthopedic surgeon, a physical
therapist, and a social worker.
• The management of MMC traditionally involves surgery within 48 h of birth. The
child’s back is closed to minimize the risk of ascending infection that can result in
meningitis.
• An earlier intervention involving fetal surgery has now been implemented in a
number of centres
• Subsequent serial head measurements to assess the velocity of head growth and
the need for shunting.
• Virtually all neonates with thoracic level lesions need a ventriculo-peritoneal
shunt, whereas around 85% of patients with a lumbar level lesion, and about 70%
with a sacral lesion, require shunting.
• In severe cases, posterior fossa decompression surgery is indicated
• Orthopedic deformities are usually treated shortly after birth, with long-term follow-up.
• Patients are also monitored by ultrasonography and urodynamic studies to detect
urological complications resulting from abnormal neurological bladder function
• Bladder and urinary tract management often includes a combination of clean intermittent
catheterization, pharmacological agents, and surgery.
• Surgical procedures include the following:
 Closure of the defect over the spinal cord
 Spinal deformity reconstruction
 Lower-extremity deformity correction
PHYSIOTHERAPY MANAGEMENT

MANUAL MUSCLE TESTING

• The role of the physical therapist can begin in the early preoperative period before back
closure with an assessment of the neonate’s active lower extremity movement.
• The therapist should tickle or stroke the baby above the level of the lesion or around the neck
and face as a stimulus to keep the baby awake and moving.
• Movement of the legs can be observed and contractions palpated by stabilizing the limb
proximally.
• Proper limb stabilization is necessary to avoid misinterpreting the origin of a movement.
• The principles for muscle testing in the infant population are much the same as those for
the older patient; gentle resistance to movement at one part of the leg may help increase
the strength of a movement at a distal part of the limb, and allowing movement to occur
at only one joint at a time will assist in a more accurate interpretation.
• Results of early manual muscle tests should be compared with later tests in order to
monitor the child’s neuromuscular stability. It is a pleasant surprise to find increased
movement or strength after back closure
RANGE OF MOTION

• Assessment of range of motion (ROM) of the lower extremities can also be performed
prior to back closure. Typical neonates have flexion contractures of up to 30 degrees at
the hips and 10 to 20 degrees at the knees, and ankle dorsiflexion of up to 40 or 50
degrees.
 POSTOPERATIVE PHYSICAL THERAPY
• The infant who has undergone back closure and shunt insertion, consideration must be given to both
the neurologic and orthopedic findings.

RANGE-OF-MOTION EXERCISES
• Daily sessions for lower extremity ROM exercises can begin after back closure and taught to parents
as soon as feasible. Passive ROM exercises should be brief and performed only two or three times
each day.
 POSITIONING AND HANDLING
• Finding a comfortable chair is most important, and once seated, the therapist or
family member can hold the child prone over their lap, rocking or swaying slowly
side to side. This position is restful for the parent and provides novel movement
for the infant.
• The baby may also enjoy a slow walk around the hospital floor while being held
up and slightly over the parent’s shoulder. This position gives the infant an
opportunity to attempt to raise his or her head and look around.
• If the supine position is contraindicated, parents may gently cradle the infant prone
across one forearm as they walk or sit.
SENSORY ASSESSMENT

• This assessment can also identify the areas of intact sensation on the baby’s trunk
and legs so stimulation at those areas will make the baby move.
• Families must always test the temperature of bath water prior to immersing the child.
• Application of new shoes or braces, for example, requires vigilance to avoid
pressure areas, sores, and abrasions
• Prior to placing the infant on the floor to play, a search for hidden objects in the
carpet may prevent an accidental injury from loose carpet tacks or a small sharp toy.
ONGOING CONCERNS AND ISSUES
Orthotics
Considered the children with
• Thoracic level lesions in one group,
• Those with high lumbar L-1 to L-3 lesions in a second group,
• Children with low lumbar L-4 to L-5 lesions in a third group, and
• Those with sacral lesions in a fourth final group.

• Early splinting, standing devices, and bracing for initial ambulation will be discussed for
each of the groups.
• One should be aware that within each group children will have very different patterns of
active movement, strengths, and upright function.
• CHILDREN WITH THORACIC-LEVEL PARALYSIS
• CHILDREN WITH HIGH LUMBAR PARALYSIS

• Children with a motor level from L-1 to L-3 will usually exhibit some amount of active
hip flexion and adduction but no other strong movements at the hips or knees are present.
• Weak quadriceps may be noted in those children with an L-3 motor level.
• To prevent flexion/adduction contractures at the hips, the child with a high lumbar lesion
will also benefit from the use of the total contact orthosis.
• The splint can maintain hip and knee extension with moderate abduction (approximately
30 degrees) and be worn during sleep. It can also serve as the child’s first standing device
• ORTHOTICS FOR CHILDREN WITH THORACIC AND HIGH LUMBAR
PARALYSIS
“A frame,” also known as the Toronto standing frame
• As the child outgrows the frame, the parapodium or the Orlau swivel walker may be
considered
• ORTHOTICS FOR CHILDREN WITH LOW LUMBAR PARALYSIS
• ORTHOTICS FOR CHILDREN WITH SACRAL-LEVEL PARALYSIS
• Casting Following Orthopedic Surgery
• HIP SPICA