Hatem I. BadrHatem I. Badr MD.MD.

ASS. Professor NeurosurgeryASS. Professor NeurosurgeryDepartment of NeurosurgeryDepartment of Neurosurgery

Mansoura University, EgyptMansoura University, Egypt

CNS congenital anomaliesCNS congenital anomaliesCNS congenital anomaliesCNS congenital anomalies

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Neural Tube Neural Tube DevelopmentDevelopment

Normal embryological development Neural plate

development -16th dayCranial closure 24th

day (upper spine)Caudal closure 28th

day (lower spine)

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Spina Bifida OccultaSpina Bifida Occulta

Very mild & common form.

Very rarely causes disability.

Can only detected by x-ray or investigating a back injury.

May be associated with tethered cord

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Usually associated with skin visible signs on the back.

– Dimple

– small hair growth

– Nevus flaminous (red spot) or port wine

– Pad of subcutaneous fat

6Dimple Dimple

7Tuft of hairTuft of hair

8Dimple with nauves port wineDimple with nauves port wine

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Tethered cord Tethered cord The spinal cord could be

caught against the vertebrae

Normal cord ends at lower end of L 1

Motor weakness of lower limbs

Sphincteric problems such as inefficient bladder control.

10Autopsy of Infant with tethered cordAutopsy of Infant with tethered cord

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MeningoceleMeningoceleMeningoceleMeningocele

Least common form Least common form

Sac contains meninges and Sac contains meninges and cerebro-spinal fluid. cerebro-spinal fluid.

Cerebro-spinal fluid protects Cerebro-spinal fluid protects the brain and spinal cord. the brain and spinal cord.

The nerves are not badly The nerves are not badly damaged and able to function damaged and able to function normally. normally.

Limited disability is presentLimited disability is present. .

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MyelomeningoceleMyelomeningoceleMyelomeningoceleMyelomeningocele Most serious and common Most serious and common

The cyst not only contains The cyst not only contains meninges and c.s.f but also the meninges and c.s.f but also the

nerves and spinal cord.nerves and spinal cord.

The spinal cord is damaged or The spinal cord is damaged or not properly developed not properly developed resulting in motor and sensory resulting in motor and sensory deficit. deficit.

Majority have bowel and Majority have bowel and

bladder problems.bladder problems.

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MyelomeningoceleMyelomeningocele

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Intact MylomeningoceleIntact Mylomeningocele

Thin transparent membrane

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Intact MylomeningoceleIntact Mylomeningocelecovered by thin membranecovered by thin membrane

surrounded by hyper pigmentationsurrounded by hyper pigmentation

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Financial CostsFinancial Costs:: Average estimated lifetime cost of $532,000 for each infant born with spina bifida (CDC 1999) adds an estimated 19 million dollars every year to Missouri resident lifetime costs associated with spina bifida.

Physical CostsPhysical Costs:: Possible paralysis (the leading cause of childhood paralysis), bowel and bladder control problems, learning disabilities, hydrocephalus, surgical procedures, latex allergies, increased health problems with age

Emotional CostsEmotional Costs:: Miscarriage, stillbirth, infant mortality (death before 1st birthday), disability, feeling “different”

The High Cost of NTDsThe High Cost of NTDs

How do you get diagnosis? How do you get diagnosis?

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preNatal detection of NTDpreNatal detection of NTD

Serum alpha-fetoprotein (AFP)Serum alpha-fetoprotein (AFP)Normal fetal glycoprotein (MW= 70,000)Present normally in amniotic fluid and mother

serum start 12 week increase steadily till 32 week

High maternal serum AFP > 2 multiples of median for appropriate week of gestation is diagnostic

91% sensitivity in spina bifida

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UltrasoundUltrasound

Detect 90-95 % of cases of spina bifida 100% cases of anencephaly

In cases of elevated AFP diffrentiate NTD fron non-neurological causes of elevated AFP

e.g. omphalocele

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AmniocentesisAmniocentesis

Indication:

Pregnancies subsequent to NTD

Elevated AFP with normal US

Show elevated AFP between 12-15 week earlier than serum AFP

Carries 6% risk of abortion and fetal loss

Is prophylaxis feasible?Is prophylaxis feasible?

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Factors Associated With Factors Associated With Increased Risk of NTDs. . .Increased Risk of NTDs. . .Factors Associated With Factors Associated With

Increased Risk of NTDs. . .Increased Risk of NTDs. . . Family history of NTD

A previous pregnancy affected with NTD

Maternal insulin-dependent diabetes

Maternal obesity

Anti-epileptic drugs (Valporic Acid, Carbamazapine)

Lower socioeconomic/educational level, dietry deficiency specially folic acid

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The only most significant risk factor associated with NTDs is folic acid deficiency

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Folic Acid For WomenFolic Acid For WomenFolic Acid For WomenFolic Acid For Women As NTD occur before diagnosis

of pregnancy.

All women of childbearing age should receive 400 micrograms (0.4 mg) of folic acid daily.

Women who have had a previous child with NTD should receive 4000 micrograms (4 mg) of folic acid daily.

What is the proper management?What is the proper management?

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Nursing CareNursing Care

Like any other neonate with congenital anomalies efforts should be towards careful examination and investigations to rule out other anomalies.

Nursed in Trendlenburg position aiming to reduce pressure and keep it away from cystic lesion.

Much care not to disturb intact membrane (high incidence of infection and urgent surgery).

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Cover lesion with Gauze ring soaked with normal saline or Ringer solution to prevent dryness

Avoid antiseptics e.g betadine as it is Neurotoxic affecting functioning roots in placode

Avoid mechanical trauma to placode

no need for ultra frequent dressing

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General assessmentGeneral assessment Assess whether lesion is ruptured or unruptured

– Ruptured lesions start prophylactic antibiotic– Urgent surgery

Measure size and site of defect for proper planing for closure

Evaluation by neonatologist – Other anomalies (average 2-2.5% additional anomalies)– Condition oppose with surgery e.g lung immaturity

Bladder – Start with on regular urinery catheterization– Urological consultation

Orthopedic consultation for sever kyphotic or scoliotic deformities and hip, knee and foot deformities

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Neurological preop. AssessmentNeurological preop. Assessment Watch for spontaneous movement of lower limbs which

associated with better outcome. Assess lowest level of neurological function

– Response to painful stimuli– Differentiate between voluntary movement from reflex

movement which is stereotyped and not persist after stimulus

Evaluate other neurological associations– Hydrocephalus

Anterior fontanel Head circumference

– Chiari II Check for inspiratory stridor and apneic episodes

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Intra-operativeIntra-operative

Cauda equina Dural edge

placode

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Post-operativePost-operative

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Baby Samuel Armas's tiny fingers grasped the Baby Samuel Armas's tiny fingers grasped the doctor's huge hand - - not at birth but, at 21 weeksdoctor's huge hand - - not at birth but, at 21 weeksduring surgery for repair of MMduring surgery for repair of MMSamuel Armas Photo 8/19/99; Born 12/2/99Samuel Armas Photo 8/19/99; Born 12/2/99

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Sara Switzer Photo: July 1Sara Switzer Photo: July 1stst /99; Born August 22 /99; Born August 22ndnd /99 /99 BORN TWICEBORN TWICE

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HydrocephalusHydrocephalus

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