SPINA BIFIDA
SPINA BIFIDA
Failure of fusion of the vertebral arches.
This mesodermal defect may be
associated with a defect of ectoderm
and neuroectoderm.
SPINA BIFIDA
First described by Nicholas Tulp 1593
Morgagni linked Hydrocephalus with
Spina Bifida 1761
TYPES
1.
Spina Bifida Occulta
20% of N. Child
2.
Spina Bifida Cystica
2.5 / 1,000
Meningocoele
- Protrusion of Meninges
Myelomeningocoele
- Protrusion of Meninges plus neural tissue
Spina Bifida Occulta
1.
2.
3.
Midline defect of Vertebral Bodies.
Hair, Lipoma, dermal sinus,
discolouration of skin.
Occasionally associated with
Diastematomyelia, Syringomyelia and
Tethered Cord.
Encephalocele
Cranial Meningocoele – Meningeal Sac
only
Cranial Encephalocele – Sac plus
Cerebral cortex, Cerebellum, Brain Stem
Anencephaly
Absent cerebral hemispheres and
cerebellum
Incid.
1/1000 live births
Neuronal Migration Disorder

Lissencephaly (Agyria)

Schizencephaly

Porencephaly
Dandy Walker Malformation
1.
Cystic Expansion of 4th Ventricle in
Post Fossa.
2.
90% have hydrocephalus.
3.
Shunting the cystic cavity.
Proposed Aetiologies for Spina Bifida
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Multi-factorial inheritance.
Potato blight.
Vitamin deficiencies/folate.
Maternal fever.
Zinc deficiency.
High sound intensity.
Viral infection.
Alcohol.
Mineral deficiency.
Medication – Phenytoin, Epilim, etc.
Spina Bifida
Incidence 2 – 2.5 / 1,000 births
Genetic Predisposition
10% incidence of positive family histories
5% incidence of Spina Bifida / Encephalocele
in sibling of an affected child
10-15% incidence if two siblings affected
Adverse Criteria
1.
2.
3.
4.
5.
6.
Gross paralysis of the legs.
Thoraco-lumbar or thoraco-l/sacral
lesions.
Kyphosis or scoliosis.
Hydrocephalus.
Intracerebral birth injury.
Other gross congenital anomalies.
Types of Lesions in Spina Bifida
Type I (1 / 3)
Complete loss of spinal cord
function below a certain
segmental level.
= ATONIC BLADDER
Type II (2 / 3)
Associated with interruption
of corticospinal tract.
= SPASTIC BLADDER.
Expectations of Lower Urinary Tracts
1.
2.
3.
4.
That bladder should fill to good
capacity.
Should empty to completion.
Emptying should be under voluntary
control.
That filling and emptying should not be
detrimental to renal function.
Failure to Fill Bladder
1.
2.
3.
Rise in intravesical pressure =
low compliance.
Detrusor hyperreflexia.
Incompetence of bladder neck and
distal sphincter mechanism =
sphincter weakness
incontinence
Failure to Empty Bladder
1.
2.
3.
No Detrusor Contraction.
Detrusor – Sphincter Dyssynergia.
Failure of bladder neck to open when
the detrusor contracts.
Aims of Treatment
1.
2.
3.
To preserve renal function.
To obtain efficient bladder emptying.
To achieve continence.
Anocutaneous reflex
=
Contractile Detrusor
Anal Tone Normal
=
No significant
sphincter weakness
Normal Perianal
Sensations
=
If all above present
=
Normal bladder and
urethral sensations
25% who will have
normal control
Videourodynamic Study
Bladder outline, size and capacity.
Detrusor pressure at rest and during filling and
voiding.
State of bladder neck and urethra.
Presence or absence of vesico-ureteric reflux.
Management of Urinary Incontinence
1.
2.
3.
4.
5.
6.
Manual expression of bladder.
Time training.
Indwelling catheterisation (silastic
balloon catheter).
Urinals – disposable penile sheaths.
Pads and napkins.
Clean intermittent catheterisations.
Investigations in Spina Bifida
1.
2.
3.
4.
5.
6.
7.
Regular urine checks q 1-3 / 12.
Urea and creatinine q 6 / 12.
I.V.U.’S q 2 years.
Renal function tests and renogram.
Measurement of residual urine.
Videourodynamic studies and micturating
cysto-urethrogram.
Renal ultrasound.
Management of Bowel Incontinence
Bowel Training.
1.
2 x 10 min periods/day
Diet.
Physical Assistance.
2.
3.
a)
b)
c)
Pressure
Manual evacuation
Suppositories
Laxatives e.g. Isagel & Senokot
Enemas
Problems of Management of Spina Bifida
1.
Shunt Complications :
Blocked shunts
Infected shunts
Shunt nephritis
Seizures 1 / 3
Upper limb weakness
Problems of Management of Spina Bifida
2.
Orthopaedic Management :
Hip dislocation
Knee flexion deformities
Foot deformities
Spinal deformities
Fractures
Problems of Management of Spina Bifida
3.
Urinary problems.
4.
Intellectual Development.
40-60% have learning disability
5.
Visual Defects.
6.
Endocrine dysfunction.
Problems of Management of Spina Bifida
7.
Obesity.
8.
Skin care.
9.
Psychological and psychosexual
problems.
10.
School placement.
Arnold Chiari Malformation

Malformation of the lower brain
stem and cerebellum and
herniation of these structures
through the foramen magnum.
HYDROCEPHALUS
1
Communicating - most common
Congenital/Acquired
2
Non Communicating
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•
•
•
•
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Spina Bifida
Aquaductal Stenosis
Dandy-Walker malformation(cystic
expansion of 4th ventricle in Post Fossa
Tumours
Aneurysmal Dilation of Vain of Galen
Perinatal Haemorrhage
Folic Acid Supplements
400 Microgram folic acid daily
Continue for 1st three months of
pregnancy