Spina Bifida

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Spina Bifida

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Case-basic data

• Name???
• Age 34 y/o
• G2P1( twins)
• Previous pregnant history
• Pregnancy 34 weeks with twins s/p C/S
• 2000g and 1700 g
• No other systemic disease.

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Current condition

• Pregnancy 354 weeks with twins
• Transfer from VGHTC
• One fetusspina bifida

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Spina Bifida

• The human nervous system develops from a small,
  specialized plate of cells along the back of an
  embryo.
• Early in development, the edges of this plate
  begin to curl up toward each other, creating the
  neural tubea narrow sheath that closes to form
  the brain and spinal cord of the embryo.
• the top of the tube becomes the brain and the
  remainder becomes the spinal cord.

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• This process is usually complete by the 28th day
  of pregnancy.
• But if problems occur during this process, the
  result can be brain disorders called neural tube
  defects, including spina bifida.

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What is spina bifida?

• Spina bifida, which literally means cleft
  spine, is characterized by the incomplete
  development of the brain, spinal cord, and/or
  meninges.
• Spina bifida is one of the more common birth
  defects in the United States.
• It is among the most common severe birth defects
  in the United States, affecting 1,500 to 2,000
  babies (one in every 2,000 live births) each
  year.

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the different types of spina bifida

• Occulta is the mildest and most common form in
  which one or more vertebrae are malformed.
• The name occulta, which means hidden,
  indicates that the malformation, or opening in
  the spine, is covered by a layer of skin. 
• This form of spina bifida rarely causes
  disability or symptoms. 

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• Closed neural tube defects make up the second
  type of spina bifida.
• This form consists of a diverse group of spinal
  defects in which the spinal cord is marked by a
  malformation of fat, bone, or membranes.
• In some patients there are few or no symptoms in
  others the malformation causes incomplete
  paralysis with urinary and bowel dysfunction.

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• meningocele the meninges protrude from the
  spinal opening, and the malformation may or may
  not be covered by a layer of skin.
• Some patients with meningocele may have few or no
  symptoms while others may experience symptoms
  similar to closed neural tube defects.

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• Myelomeningocele is the most severe and occurs
  when the spinal cord is exposed through the
  opening in the spine, resulting in partial or
  complete paralysis of the parts of the body below
  the spinal opening. 
•  The paralysis may be so severe that the affected
  individual is unable to walk and may have urinary
  and bowel dysfunction. 

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What causes spina bifida?

• The exact cause of spina bifida remains a
  mystery. 
• No one knows what disrupts complete closure of
  the neural tube, causing a malformation to
  develop.
• Scientists suspect genetic, nutritional, and
  environmental factors play a role. 
• it appears to result from a combination of
  genetic and environmental risk factors, such as a
  family history of neural tube defects, folic acid
  deficiency and medical conditions such as
  diabetes and obesity.

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Risk factors

• Race. Spina bifida is more common among Hispanics
  and whites of European descent.
• Family history of neural tube defects. Couples
  who've had one child with a neural tube defect
  have a slightly higher chance of having another
  baby with the same defect. That risk increases if
  two previous children have been affected by the
  condition. In addition, a woman who was born with
  a neural tube defect, or who has a close relative
  with one, has a greater chance of giving birth to
  a child with spina bifida. However, most babies
  with spina bifida are born to parents with no
  known family history of the condition.

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• Folic acid deficiency. This vitamin is important
  to the healthy development of a fetus. Lack of
  folic acid (vitamin B-9) increases the risk of
  spina bifida and other neural tube defects.
• Some medications. Anti-seizure medications, such
  as valproic acid (Depakene), seem to cause neural
  tube defects when taken during pregnancy, perhaps
  because they interfere with the body's ability to
  use folic acid.

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• Diabetes. The risk of spina bifida increases with
  diabetes, especially when the mother's blood
  sugar is elevated early in her pregnancy. Much of
  this risk is preventable by careful blood sugar
  control and management.
• Obesity. There's a link between pre-pregnancy
  obesity and neural tube birth defects, including
  spina bifida. Obese women may have more babies
  with spina bifida possibly because of nutritional
  deficits from poor eating habits or because they
  may have diabetes.

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• Increased body temperature. Some evidence
  suggests that increased body temperature
  (hyperthermia) in the early months of pregnancy
  may increase the risk of spina bifida.

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Complications

• Factors that affect the severity of complications
  include
• The size and location of the neural tube defect .
• Whether skin covers the affected area
• Whether spinal nerves come out of the affected
  area of the spinal cord.
• Children with myelomeningocele may experience
  physical and neurological problems, including
  lack of normal bowel and bladder control, and
  partial or complete paralysis of their legs.

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• Babies born with myelomeningocele also commonly
  experience accumulation of fluid in the brain, a
  condition known as hydrocephalus.
• Most babies with myelomeningocele will need a
  shunt .
• The majority of newborns with myelomeningocele
  survive.
• some may develop meningitis, an infection in the
  tissues surrounding the brain

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• Children with myelomeningocele may develop
  learning disabilities.
• Children with spina bifida may also suffer from
  latex allergies, skin problems, urinary tract
  infections, gastrointestinal disorders, seizure
  disorders, depression, and social and emotional
  problems.

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Screening and diagnosis

• (1)Blood tests
• second trimester maternal serum alpha fetoprotein
  (MSAFP)
• alpha-fetoprotein (AFP) is made naturally by the
  fetus and placenta. 
• But if abnormally high levels of this protein
  appear in the mothers bloodstream it may
  indicate that the fetus has a neural tube defect.
  
• The MSAFP test, however, is not specific for
  spina bifida.

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• Ultrasound An advanced ultrasound can also
  detect signs of spina bifida.
• AmniocentesisAn analysis indicates the level of
  AFP present in the amniotic fluid.
• A small amount of AFP is normally found in
  amniotic fluid.
• when an open neural tube defect is present, the
  amniotic fluid contains an elevated amount of AFP
  because the skin surrounding the baby's spine is
  gone and AFP leaks into the amniotic sac.
• MRI

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Treatment

• There is no cure for spina bifida.
• The nerve tissue that is damaged or lost cannot
  be repaired or replaced.
• Treatment depends on the type and severity of the
  disorder.
• children with the mild form need no treatment .

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• The key priorities for treating myelomeningocele
  are to prevent infection from developing through
  the exposed nerves and tissue of the defect on
  the spine, and to protect the exposed nerves and
  structures from additional trauma.
• Doctors have recently begun performing fetal
  surgery for treatment of myelomeningocele.

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• Fetal surgery involves opening the mothers
  abdomen and uterus and sewing shut the opening
  over the developing babys spinal cord. 
• They believe the earlier the defect is corrected,
  the better the outcome is for the baby.
• Still, the benefits of fetal surgery are
  promising, and include less exposure of the
  vulnerable spinal nerve tissue and bones to the
  intrauterine environment, in particular the
  amniotic fluid, which is considered toxic.

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• Early surgery on the spinal cord may allow the
  child to regain a normal level of functioning and
  prevent further neurological deterioration.
• Some children will need subsequent surgeries to
  manage problems with the feet, hips, or spine.
• Individuals with hydrocephalus generally will
  require additional surgeries to replace the shunt
  
• Some individuals with spina bifida require
  assistive devices such as braces, crutches, or
  wheelchairs.  The location of the malformation on
  the spine often indicates the type of assistive
  devices needed. 

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• Treatment for paralysis and bladder and bowel
  problems typically begins soon after birth

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Prevention

• Folic acid is an important vitamin in the
  development of a healthy fetus.
• Recent studies have shown that by adding folic
  acid to their diets, women of childbearing age
  significantly reduce the risk of having a child
  with a neural tube defect, such as spina bifida.
   
• Dosage400 micrograms of folic acid daily
• Foods high in folic acid include dark green
  vegetables, egg yolks, and some fruits.

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prognosis

• Prognosis depends on the number and severity of
  abnormalities and associated complications.