SPINA BIFIDA

Dr A Lubega
 Definition of Spina Bifida
• Congenital abnormality of spinal axis with a
developmental defect in spinal column with
incomplete closure of vertebral canal due to
failure of fusion of vertebral arches.

• Protrusions and dysplasia of the spinal cord or

its membranes may or may not be present
Bone appearance
• Spina bifida occulta
Differentials
– Failure of bony structures to fuse
– Cord structure and neurological function is normal
– Rarely associated with hydrocephalus
– Usu found incidentally of radiological examination of the lumbosacral area
– Tuft of hair, lipoma,cutaneous angioma, sinus tract although rare

• Meningocele
– Meninges fail to close – cystic cutaneous abnormality
– Usu no neurological deficit

• Spina bifida cystica (spina bifida aperta)

– Underlying neural tissue fails to fuse
• Myelomeningocele
– Incomplete closure of neural groove usu in lumbar region
– Associated defect in spinal column
– Covered with epithelium
– Complete absence of motor and sensory function below level
– Lesions L2 and above are usu associated with kyphois, scoliosis, dislocated hips, club feet
• Myeloschisis
– Unfused spinal cord
– No meninges or epithelium covering the defect
– Usu in thoracolumbar region
– Paraplegia and loss of bladder function
 Spina Bifida Occulta
-Occulta is Latin for "hidden
• mildest form.
There is an opening in one or more of the vertebrae of the spinal
column without apparent damage to the spinal cord. Sufferers
may even know they have it.
Evidence for this may be just

• A tuff of hair.
• The skin at the site of the lesion may be normal,
• There may be a dimple in the skin,
• A lipoma, lipomyelomeningocele
• A dermal sinus
• A birthmark.
• (diastematomyelia) when the spinal cord gets trapped below
the affected level of the growing spine
• Thickened filum tarminale
Spina Bifida Occulta:
Contrast media appearance

Spina Bifida Occulta radiograph

Visible swelling in Spina Bifida
• Incidence 5%-36%
• No hydrocephalus
• May be no neurologic symptoms
• Its asymptomatic and most common in lower
lumbar spine
• Involves the laminae of L5 and S1 in most cases
• There may be spinal cord or nerve root
distortion due to fibrous bands or adhesions
causing tethering of spinal cord.
 Common associated findings
• Decreased achilles tendon reflexes
• Shortened calf muscles
• Talipes Equinus Varus
• Unequal leg length
 Meningocele
The meninges, or protective covering around the spinal cord, has
pushed out through the opening in the vertebrae in a sac called
the "meningocele."
• Less than 10%
• The spinal cord remains intact. There are two types:

• Posterior meningocele - meningeal cyst, the outer faces of

some vertebrae are open (unfused) and the meninges are
damaged and pushed out through the opening, appearing as
a sac or cyst which contains cerebrospinal fluid. The spinal
cord and nerves are not involved and their function is normal.

• Anterior meningocele: the inner faces of vertebrae are

affected and the cyst protrudes into the retroperitoneal or the
presacral space.
Meningocele:
 Myelomeningocele:
• This is the most severe form of spina bifida, in which a
portion of the spinal cord itself protrudes through the
vertebrae.
• In some cases, sacs are covered with skin, in others
tissue and nerves are exposed.
• Forms the majority
• Hydrocephalus in 90%
• Involves spinal cord, nerve roots and meninges that
protrude thru vertebral defect.
• Motor/sensory deficit, bladder and bowel involvement
present.
• 75% of the lesion at lumbar level
Myelomeningocele:
 Risk factors and Etiology
• Sporadic versus multifactorial inheritance Causes, contribute,
correlate.
• Teratogenic agents act prior to the fourth gestational week when
closure of neural tube normally occurs.
• Vitamin A: ( animal liver, cheese, cream, egg yolk, carrots,
tomatoes) too much, causes clefting in humans. This refers to the
preformed vit A (animal products and vit A suppliments). Pro vit A
carotenoids in vegetable does not have this toxicity
• Folic acid: (a Vit B found in dark green vegetables, citrus fruits,
kidney, liver) too little, causes clefting in humans.
• Smoking: some people have a gene (A2 TGFa-Transforming
Growth Factor-alpha gene) when exposed to cigarette smoke will
result in clefts. Cigarette smoke affects folic acid levels and
probably CO and nicotine may lower the Oxgyen available to
featus.
 Effects of Wars
• Gulf war syndrome in children born to Gulf war
veterans.

• We do not know the health effects yet from

Uganda Somalia/Iraq/world war 1/world
war2/Luweero/LRA/ Lakwena wars etc.

• History might find similar effects as those of Gulf

wars
• There is increased risk with subsequent births
once one infant is born with SB
• Rate of occurrence is about 1:1000 where no
other family member is affected
• 5.5:100 after one affected child
• 13:100 after two affected children
• 20.6:100 after three affected children
• Female to male ratio: 1.2:1 (Garrison, 2003)
 Other risk factors
• Low social economic status
• Maternal obesity
• Heat exposure/maternal febrile illness
• High environmental temps
• Low folic acid intake
• Use of anticonvulsants such as valproic acid &
carbamazepine
 Clinical features in Myelomeninocele
• Flaccid paralysis
• Muscle weakness
• Muscle wasting
• Decreased or absent tendon reflexes
• Decreased or absent exteroceptive and
proprioceptive sensation
• Rectal & bladder incontinence
• Paralytic and congenital deformities
• hydrocephalus
 Secondary clinical feature include
• Pressure ulceration of skin due absent
sensation and poor skin nutrition
• Osteoporosis with probability of fractures
• Retarded mental, physical and emotional dev’t
due to failure to move & explore the
environment, play & interact with other
children.
• Contractures & deformity due to unopposed
mms action, gravity & posture
Conditions associated with Spina Bifida
a) Hydrocephalus. About 70 to 90 percent of
children with Myelomeningocele develop
hydrocephalus but in developing world this is
less.

When cerebrospinal fluid, which cushions and

protects the brain and spinal cord, is unable
to circulate normally, fluid collects in and
around the brain, causing the head to be
enlarged.
 Associated conditions contd
Arnold Chiari II malformation.
The lower part of the brain is located further down
than normal and is partly displaced into the upper part
of the spinal canal, blocking the flow of CSF and
contributing to hydrocephalus in 20% and
compression.
• .In most cases, affected children have no other
symptoms.
• A small number develop serious problems, such as
breathing and swallowing difficulties and upper
body weakness.
 Associated conditions contd

Tethered spinal cord. Most children with

Myelomeningocele, and a small number with
meningocele or most of spina bifida occulta,
have a tethered spinal cord.
• The spinal cord does not slide up and down with
movement as it should, because it is held in place
by surrounding tissue.
• Some children have no symptoms, but others
develop leg weakness, worsening leg function,
scoliosis , pain in the back or legs and changes in
bladder function.
 Associated conditions contd
Urinary tract disorders.
• Individuals with myelomeningocele often have problems
emptying the bladder completely. This can lead to urinary
tract infections and kidney damage.

Latex allergy.
• According to the Spina Bifida Association of America (SBAA),
up to 73% of children with myelomeningocele are allergic to
latex-natural rubber, possibly due to repeated exposures
during surgeries (catheterization/gloves) and medical
procedures. Symptoms may include watery eyes, wheezing,
hives, rash and even life-threatening anaphylactic reactions.
 Associated conditions contd
• Learning disabilities. At least 80% of children
with myelomeningocele have normal
intelligence. However, some have learning
problems.

• Other conditions associated with severe spina

bifida include obesity, digestive tract disorders
and psychological and sexual issues
 Associated conditions
• Musculoskeletal system including ribs, spinal
cord and limbs.
• cardiac,
• congenital scoliosis wedge vertebra
• butterfly vertebra
• bloc vertebra.
• karyotypic abnormalities .
• VACTERL syndrome
 Other associated congential
abnormalities
• Dislocated hips
• TEV
• Hemi vertebrae with resultant scoliosis
• Local kyphosis due to vertebral anormaly
• Cleft palate
• Cardiac & urinary tract abnormalities
 Social & pyschological factors
• There is an atmosphere of sorrow at the birth
of the child with SB
• Parents develop a feeling of resentment or
guilt or a sense of inadequacy with regard to
problems ahead
• Parents here need education & counseling
services
 Practical problems for parents
• Expense of medical and hospital care
• Schooling and habilitation of the child
• Fear of having another affected child
• Neglect of other children in the home
spending more time with disabled child
• Profound stress on whoever comes in contact
with the child with SB
• Child’s ability to explore the environment is
impaired by :
• Difficulty in achieving upright postns (sitting
and standing)
• Inability to move about and play
• Child can be neglected by parents who cannot
cope with the situation that demands a great
deal from them.
• This affects child physically and mentally.
• Children with SB are:
• less adaptable to stresses and strains
• tend to be emotionally immature
• Have low self-esteem
• Have decreased opportunity for interaction
with peers
• Have unreliable bowel & bladder function due
to overflow incontinence
• There is no sensation of fullness due to lack of
sensory feedback from denervated bladder ----
cause of UTIs
• Have fewer chances of being given household
responsibility which may all be obstacles for
emotional growth
 Initial Management of SB
• The overall management of SB is lengthy and
multidesciplinary.
• It’s a complete package
• It begins with closure of the spinal defect
 The ultimate goal
In the care of a child with Spina Bifida is:

To achieve the greatest possible degree of self-

reliance and dignity- both for the disabled child
and his or her family.
 Also involves the following

1. Musculoskeletal problems
2. Other neurological problems
3. Gastrointestinal problems
4. Urological = most challenging and main
cause of morbidity
5. Physiotherapy
6. Occupation therapist
 Factors to Consider

1. Contraindications to surgery
Renal agenesis
Lung agenesis

2. Selective approach
3. Small menigoceles of children who come
Late
 Factors to consider continued

1. Timing of closure
2. Antibiotic regime
3. Counseling to parent/child care givers
4. Other problems which need treatment
e.g (i) Hydrocephalus
(ii) Orthopedic problems
 Surgery

Goals of Techniques

1. Seal the spinal cord

2. Prevent entrance of bacteria from the skin
3. Prevention of CSF leakage
4. Preserve the existing neurological function
5. Prevent tethering of the spinal cord
 Operative Technique

Picture
 Approach
Patient position
 Steps of standard repair of an open S.B

1. Incision of the sac or of the skin surrounding the placode

2. Circumferential isolation of the placode with removal of all keratinized
areas as well as the superficial granulation tissue
3. Tubularization of the placode
4. Lysis of all adhesins to the cord both proximally and distally
5. Circumferential disection of dura
6. Water tight dural closure
7. Wide undermining of skin and subcutaneous tissue laterally
8. “Fascial” closure
9. Skin closure
 POST OPERATIVE CARE
Keep patient off all incisions
Bladder catherisation regimes
Daily OFC measurement
If not shunted
• regular head u/s (twice weekly to weekly
• Keep patient flat to reduce CFS pressure on
incision
 Post operative complications

Late complications
1. Wound problems
Infection 1. Urological
Dehiscence complications
Necrosis 2. Re-tethering of the
2. CSF leaks cord at the repair site
3. Hydrocephalus 15-20% cares
3. Chest problems
 Urological management
Spine bifida is the main cause of neurogic dysfunction which
account for the main cause of mortality.
Urological complications:
• Vesico-ureteric reflux (VUR)
• Renal failure
• Chronic urinary tract infection

Types of dysfunction
I Synergic = 19%
II Dysynergic (DSD) 45%
III Denervated (36%
 Evaluation

I) Ultrasound

II) Serum creatine

III) Volume urodynamics

a) Leak-point pressure (LPP)
b) Post-void residual volumes (PVR)
 Treatment

1. Clean Intermittent catherisation (CIC)

2. Medication
3. Surgery
 Clean Intermittent catherization (CIC)

Advantages CIC, Later – CISC

• Simple
• Cheap
• Safe
• Very efficient
• Easy to teach
• Well suited for resource – poor societies
CIC continued

Indications
LPP = 30cm or
• PVR = 10cc
Other indicators
• Laboratory evidence of renal dysfunction
• Recurrent urinary tract infections
• Need to promote social continence in older
children
CIC continue

Effect Of CIC And Medication

• Prevention of renal deterioration in 90%
cases
• Social continence in 85% cases
 Surgery

Only for the monitory of patients

• Bladder augmentation
• Bladder neck reconstruction
• Urinary diversion
• Renal transplant
 Goals for rehabilitation
• To promote mobility
• Promote independent self-care (including
neurologic bladder and bowel mgt at age-
appropriate time)
• Prevent secondary conditions
• Determine adaptations for educational &
vocational issues
 Function of the Physiotherapist
• To promote functional independency by:
• Preventing adaptive deformity
• Preventing dev’t of pressure ulcers
• Correction of deformity already present
• Promotion of learning of critical motor skills
e.g postural adjustment e.g in sitting &
standing
• Strengthening exs of arm, trunk and lower limb mms.
• Indepedent ambulation and where necessary train
wheel chair activities.
• Bladder and bowel training.
• Assessment, measuring and fitting of appropriate
orthoses essential for standing and ambulation
• Teaching parents home programs
 Exercises employed by the PT
• Passive mobilisation of joints thru ROM
• Passive stretching of tight soft tissue
structures
• Increased stimulation of the child in motor
and sensory training
• Training balance in sitting, standing and
ambulation using orthoses
 OURS
O – Organized
U – Useful
R – Rehabilitation
S - Service
What does OURS offers for children with
Spina Bifida

1. Counseling for parents

2. Parent Support Group
2. Physiotherapy/Occupationaltherapy
3. Clean Intermittent Catheterization
4. Collaboration with CURE Hospital in
Mbale
THANKS FOR LISTENING!