congenital and developmental treatment of spina bifida,tethered cord and syringomyelia

1. A N D R E A
Developmental disease of
spinal cord

2. Embryology
 Human nervous system develops from a
specialized plate of cells along the back of the
embryo
 Early in the development the edges of this
plate begin to curl up towards each other
creating the neural tube
 Anterior end becomes brain and the posterior
become spinal cord.Complete by 28th day of
pregnancy

4. Spina Bifida
 SPLIT/CLEFT SPINE
 Most common NTD
 In lumbosacral region
 1/1000 births

5.  What causes spina bifida?
 Risk factors
 Pathogenesis
 Types-spina bifida cystica(open type)
spina bifida occulta(closed type)

6. Spina bifida apperta
 Myelocele-spinal cord is exposed so that the
nerve tissue lies exposed on the surface of the
back without even covering of meninges
 Myelomeningocele-neural placode and
meninges protrude above the skin.Almost
always seen with Arnold Chiari 2 malformation
 Hemimyelocele-myelocele+diastematomyelia
 Hemimyelomeningocele

7. Spina bifida occulta
 Often unnoticed
 It can be associated with other conditions that
could lead to problems with movements and
bladder control
 Meningocele
 Lipomyelomeningocele
 Dermal sinus
 Diastematomyelia

10. Diagnostic evaluation
 Neural tube defects can usually be detected
during pregnancy by testing the mother's
blood (AFP screening) or a detailed
foetal ultrasound.
 Increased levels of maternal serum alpha-
fetoprotein (MSAFP) should be followed up by
two tests - an ultrasound of the foetal spine
and amniocentesis (to test for alpha-
fetoprotein and acetylcholinesterase).

12. Treatment
 There is no known cure for nerve damage
caused by spina bifida.
 The spinal cord and its nerve roots are put back
inside the spine and covered with meninges.
 In addition, a shunt may be surgically installed
to provide a continuous drain for the excess
cerebrospinal fluid produced in the brain, as
happens with hydrocephalus.
 Shunts most commonly drain into
the abdomen or chest wall.

13.  Monitor growth and development
 Treat and evaluate nervous system issues, such
as seizure disorders.
 Physical therapy
 Speech therapy

14. Immediate Treatment
 Place the child in prone position.
 Cover the affected area with sterile gauze piece
dipped in normal saline.
 Maintain hydration.
 Monitor for associated defects.

15. Neurological
disorder
caused by
tissue
attachments
that limit the
movements
of spinal
cord within
spinal
column.
Tethered cord
syndrome

16. causes Signs and symptoms
 Myelomeningocele
 Lipomyelomeningoce
le
 Dermal sinus
 Diastematomyelia
 Tumor
 Lesion on lower back
 Fatty tumor or deep
dimple
 Hairy patch
 Back pain
 Leg pain and numbness
 Leg deformity
 Spine tenderness
 Scoliosis
 Bowel and bladder
problems

17. Diagnosis
MYELOGRAM
USG
CT
MRI

18. TREATMENT
 If the patient has predominantly back pain and
mild weakness, a course of physical therapy
may provide tethered cord treatment.
 In most cases, surgical treatment of tethered
cord is needed to prevent neurologic
deterioration. A laminectomy is performed, the
dura is opened and using the operating
microscope, the spinal cord is freed from the
tethering structure.

19. Syringomyelia
 Development of tubular CSF filled cavity in the
substance of spinal cord
 Can be congenital or acquired
 Congenital-associated with Chiari 1 defect
 Present with loss of pain and temperature in
upper limb

21.  Treatment
 Chiari defect decompressed by suboccipital
craniectomy or upper cervical laminectomy
 Large syrinx cavity –direct decompression or
drainage

22. THANK YOU