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SPINA BIFIDA
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SPINA BIFIDA
Failure of fusion of the vertebral Failure of fusion of the vertebral arches. This mesodermal defect arches. This mesodermal defect may be associated with a defect of may be associated with a defect of ectoderm and neuroectoderm.ectoderm and neuroectoderm.
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SPINA BIFIDA
First described by Nicholas Tulp -
1593
Morgagni linked Hydrocephalus with Spina Bifida -
1761
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TYPES
1.1. Spina Bifida OccultaSpina Bifida Occulta20% of N. Child20% of N. Child
2.2. Spina Bifida CysticaSpina Bifida Cystica2.5 / 1,0002.5 / 1,000
MeningocoeleMeningocoele- Protrusion of Meninges- Protrusion of MeningesMyelomeningocoeleMyelomeningocoele- Protrusion of Meninges plus neural - Protrusion of Meninges plus neural tissuetissue
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Spina Bifida Occulta
1.1. Midline defect of Vertebral Bodies.Midline defect of Vertebral Bodies.
2.2. Hair, Lipoma, dermal sinus, Hair, Lipoma, dermal sinus, discolouration of skin.discolouration of skin.
3.3. Occasionally associated with Occasionally associated with Diastematomyelia, Syringomyelia Diastematomyelia, Syringomyelia and Tethered Cord.and Tethered Cord.
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Encephalocele
Cranial Meningocoele – Meningeal Cranial Meningocoele – Meningeal Sac onlySac only
Cranial Encephalocele – Sac plusCranial Encephalocele – Sac plus
Cerebral cortex, Cerebellum, Brain Cerebral cortex, Cerebellum, Brain StemStem
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Anencephaly
Absent cerebral hemispheres and Absent cerebral hemispheres and cerebellumcerebellum
Incid.Incid. 1/1000 live births1/1000 live births
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Neuronal Migration Disorder
Lissencephaly (Agyria)Lissencephaly (Agyria)
SchizencephalySchizencephaly
PorencephalyPorencephaly
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Dandy Walker Malformation1.1. Cystic Expansion of 4Cystic Expansion of 4thth Ventricle Ventricle
in Post Fossa.in Post Fossa.
2.2. 90% have hydrocephalus.90% have hydrocephalus.
3.3. Shunting the cystic cavity.Shunting the cystic cavity.
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Proposed Aetiologies for Spina Bifida Multi-factorial inheritance.Multi-factorial inheritance. Potato blight.Potato blight. Vitamin deficiencies/folate.Vitamin deficiencies/folate. Maternal fever.Maternal fever. Zinc deficiency.Zinc deficiency. High sound intensity.High sound intensity. Viral infection.Viral infection. Alcohol.Alcohol. Mineral deficiency.Mineral deficiency. Medication – Phenytoin, Epilim, etc.Medication – Phenytoin, Epilim, etc.
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Spina Bifida
Incidence 2 – 2.5 / 1,000 birthsIncidence 2 – 2.5 / 1,000 births
Genetic PredispositionGenetic Predisposition
10% incidence of positive family histories10% incidence of positive family histories5% incidence of Spina Bifida / 5% incidence of Spina Bifida /
Encephalocele Encephalocele in sibling of an affected childin sibling of an affected child
10-15% incidence if two siblings affected10-15% incidence if two siblings affected
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Adverse Criteria
1.1. Gross paralysis of the legs.Gross paralysis of the legs.
2.2. Thoraco-lumbar or Thoraco-lumbar or thoraco-l/sacral lesions.thoraco-l/sacral lesions.
3.3. Kyphosis or scoliosis.Kyphosis or scoliosis.
4.4. Hydrocephalus.Hydrocephalus.
5.5. Intracerebral birth injury.Intracerebral birth injury.
6.6. Other gross congenital anomalies.Other gross congenital anomalies.
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Types of Lesions in Spina Bifida
Type I (1 / 3)Type I (1 / 3) Complete loss of spinal Complete loss of spinal cord cord function below a function below a certain certain segmental level. segmental level.
= ATONIC BLADDER= ATONIC BLADDER
Type II (2 / 3)Type II (2 / 3) Associated with Associated with interruption interruption of of corticospinal tract. corticospinal tract.
= SPASTIC BLADDER.= SPASTIC BLADDER.
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Expectations of Lower Urinary Tracts
1.1. That bladder should fill to good That bladder should fill to good capacity.capacity.
2.2. Should empty to completion.Should empty to completion.
3.3. Emptying should be under Emptying should be under voluntary control.voluntary control.
4.4. That filling and emptying should That filling and emptying should not be detrimental to renal not be detrimental to renal function.function.
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Failure to Fill Bladder
1.1. Rise in intravesical pressure = Rise in intravesical pressure = low low
compliance.compliance.2.2. Detrusor hyperreflexia.Detrusor hyperreflexia.3.3. Incompetence of bladder neck and Incompetence of bladder neck and
distal sphincter mechanism = distal sphincter mechanism = sphincter weakness sphincter weakness incontinenceincontinence
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Failure to Empty Bladder
1.1. No Detrusor Contraction.No Detrusor Contraction.
2.2. Detrusor – Sphincter Dyssynergia.Detrusor – Sphincter Dyssynergia.
3.3. Failure of bladder neck to open Failure of bladder neck to open when the detrusor contracts.when the detrusor contracts.
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Aims of Treatment
1.1. To preserve renal function.To preserve renal function.
2.2. To obtain efficient bladder To obtain efficient bladder emptying.emptying.
3.3. To achieve continence.To achieve continence.
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Anocutaneous reflexAnocutaneous reflex == Contractile DetrusorContractile Detrusor
Anal Tone NormalAnal Tone Normal == No significant No significant sphincter weaknesssphincter weakness
Normal Perianal Normal Perianal Normal bladder and Normal bladder and SensationsSensations == urethral sensationsurethral sensations
If all above presentIf all above present == 25% who will have 25% who will have normal controlnormal control
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Videourodynamic Study
Bladder outline, size and capacity.Bladder outline, size and capacity.
Detrusor pressure at rest and during filling Detrusor pressure at rest and during filling and voiding.and voiding.
State of bladder neck and urethra.State of bladder neck and urethra.
Presence or absence of vesico-ureteric Presence or absence of vesico-ureteric reflux.reflux.
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Management of Urinary Incontinence
1.1. Manual expression of bladder.Manual expression of bladder.
2.2. Time training.Time training.
3.3. Indwelling catheterisation (silastic Indwelling catheterisation (silastic balloon catheter).balloon catheter).
4.4. Urinals – disposable penile sheaths.Urinals – disposable penile sheaths.
5.5. Pads and napkins.Pads and napkins.
6.6. Clean intermittent catheterisations.Clean intermittent catheterisations.
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Investigations in Spina Bifida1.1. Regular urine checks q 1-3 / 12.Regular urine checks q 1-3 / 12.
2.2. Urea and creatinine q 6 / 12.Urea and creatinine q 6 / 12.
3.3. I.V.U.’S q 2 years.I.V.U.’S q 2 years.
4.4. Renal function tests and renogram.Renal function tests and renogram.
5.5. Measurement of residual urine.Measurement of residual urine.
6.6. Videourodynamic studies and Videourodynamic studies and micturating cysto-urethrogram.micturating cysto-urethrogram.
7.7. Renal ultrasound.Renal ultrasound.
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Management of Bowel Incontinence
1.1. Bowel Training.Bowel Training.2 x 10 min periods/day2 x 10 min periods/day
2.2. Diet.Diet.
3.3. Physical Assistance.Physical Assistance.a)a) PressurePressure
b)b) Manual evacuationManual evacuationc)c) Suppositories Suppositories
Laxatives e.g. Isagel & SenokotLaxatives e.g. Isagel & Senokot
EnemasEnemas
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Problems of Management of Spina Bifida
1.1. Shunt ComplicationsShunt Complications ::
Blocked shuntsBlocked shunts
Infected shuntsInfected shunts
Shunt nephritisShunt nephritis
Seizures 1 / 3Seizures 1 / 3
Upper limb Upper limb weaknessweakness
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Problems of Management of Spina Bifida
2.2. Orthopaedic ManagementOrthopaedic Management ::
Hip dislocationHip dislocation
Knee flexion Knee flexion deformitiesdeformities
Foot deformitiesFoot deformities
Spinal deformitiesSpinal deformities
FracturesFractures
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Problems of Management of Spina Bifida
3.3. Urinary problems.Urinary problems.
4.4. Intellectual Development.Intellectual Development.40-60% have learning disability40-60% have learning disability
5.5. Visual Defects.Visual Defects.
6.6. Endocrine dysfunction.Endocrine dysfunction.
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Problems of Management of Spina Bifida
7.7. Obesity.Obesity.
8.8. Skin care.Skin care.
9.9. Psychological and psychosexual Psychological and psychosexual problems.problems.
10.10. School placement.School placement.
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Arnold Chiari Malformation
Malformation of the lower Malformation of the lower brain stem and cerebellum brain stem and cerebellum and herniation of these and herniation of these structures through the structures through the foramen magnum.foramen magnum.
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HYDROCEPHALUS11 Communicating - most common Communicating - most common
Congenital/AcquiredCongenital/Acquired
22 Non CommunicatingNon Communicating• Spina BifidaSpina Bifida• Aquaductal StenosisAquaductal Stenosis• Dandy-Walker malformation(cystic Dandy-Walker malformation(cystic
expansion of 4expansion of 4thth ventricle in Post Fossa ventricle in Post Fossa• TumoursTumours• Aneurysmal Dilation of Vain of GalenAneurysmal Dilation of Vain of Galen• Perinatal HaemorrhagePerinatal Haemorrhage
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Folic Acid Supplements
400 Microgram folic acid daily400 Microgram folic acid daily
Continue for 1Continue for 1stst three months of three months of pregnancypregnancy