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Spina bifida

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jannet reena
jannet reena

Spina bifida is a birth defect where the spinal cord fails to develop properly, leaving an opening in the vertebrae. It occurs when the neural tube fails to close fully during early embryonic development. There are three main types: spina bifida occulta, meningocele, and myelomeningocele. Myelomeningocele is the most severe form and often results in paralysis and loss of sensation below the level of the defect. Risk factors include family history, obesity, fever during early pregnancy, and folic acid deficiency. Treatment involves surgery to close the opening and address any related issues like hydrocephalus. Lifelong management focuses on rehabilitation, preventing infections and complications, and addressing issues with mobility,

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SPINA BIFIDA PRESENTED BY: JANNET REENA PURANI
DEFINITION:  Spina bifida (Latin: "split spine") is a developmental congenital disorder caused by the incomplete closing of the embryonic neural tube.  Some vertebrae overlying the spinal cord are not fully formed and remain unfused and open.  If the opening is large enough, this allows a portion of the spinal cord to protrude through the opening in the bones.  There may or may not be a fluid-filled sac surrounding the spinal cord.
INCIDENCE:  Spina bifida is one of the most common birth defects, with an average worldwide incidence of one to two cases per 1000 births, but certain populations have a significantly greater risk.  Myelomeningocele is the most significant and common form, and this leads to disability in most affected individuals.  This condition is more likely to appear in females; the cause for this is unknown.
RISK FACTORS • Race: morecommonamongHispanicsand whitesof Europeandescent. • Family history of neural tube defects. • However,mostbabieswithspinabifidaareborn toparentswithno knownfamilyhistoryofthe condition. • Folic acid deficiency (vitamin B-9) • Increased body temperature. Someevidence suggests that increased body temperature (hyperthermia)inthe early months ofpregnancy may increasetheriskofspinabifida.
CAUSES:  Maternal diabetes  Family history  Obesity  Increased body temperature from fever or external sources such as hot tubs and electric blankets may increase the chances of delivery of a baby with a spina bifida.  Medications such as some anticonvulsants.  Pregnant women taking Valproic acid have an increased risk of having children with spina bifida  Genetic basis.  Folic acid deficiency
EMBRYOLOGY:  Spina bifida is caused by the failure of the neural tube to close during the first month of embryonic development (often before the mother knows she is pregnant).  Under normal circumstances, the closure of the neural tube occurs around the 23rd (rostral closure) and 27th (caudal closure) day after fertilization.
Spina bifida
TYPES:  Spina bifida malformations fall into three categories:  spina bifida occulta  spina bifida cystica with meningocele  spina bifida cystica with myelomeningocele. (The most common location of the malformations is the lumbar and sacral areas)
Spina bifida
SPINA BIFIDA OCCULTA:  Occulta is Latin for "hidden". This is the mildest form of spina bifida.  In occulta, the outer part of some of the vertebrae is not completely closed.  The splits in the vertebrae are so small that the spinal cord does not protrude.  The skin at the site of the lesion may be normal, or it may have some hair growing from it; there may be a dimple in the skin, or a birthmark.  The incidence of spina bifida occulta is approximately 10% of the population, and most people are diagnosed incidentally from spinal X- rays
MENINGOCELE:  The least common form of spina bifida is a posterior meningocele (or meningeal cyst). In this form, the vertebrae develop normally, but the meninges (pia & arachnoid) are forced into the gaps between the vertebrae. • Spinal cord is not involved. • There is no paralysis. • Sac contains only CSF • Surgical closure is done to prevent rupture and meningitis.
Spina bifida
MYELOMENINGOCELE:  This type of spina bifida often results in the most severe complications.  In individuals with myelomeningocele, the unfused portion of the spinal column allows the spinal cord to protrude through an opening.  The meningeal membranes that cover the spinal cord form a sac enclosing the spinal elements.  Spina bifida with myeloschisis is the most severe form of myelomeningocele. In this type, the involved area is represented by a flattened, plate-like mass of nervous tissue with no overlying membrane.  The exposure of these nerves and tissues make the baby more prone to life-threatening infections such as meningitis.
Spina bifida
CONTD…  The protruded portion of the spinal cord and the nerves that originate at that level of the cord are damaged or not properly developed.  As a result, there is usually some degree of paralysis and loss of sensation below the level of the spinal cord defect.
CLINICALMANIFESTATIONS: Physical Signs:  Orthopedic abnormalities (i.e., club foot, hip dislocation)  Bladder and bowel control problems, including incontinence, urinary tract infections, and poor renal function.  Pressure sores and skin irritations  Abnormal eye movement  68% of children with spina bifida have an allergy to latex  Paralysis
CONTD…  Scoliosis  Back pain  Partial or complete lack of sensation  Weakness of the hips, legs, or feet of a newborn  Other symptoms may include:  Hair at the back part of the pelvis called the sacral area  Dimpling of the sacral area  Difficulty swallowing, which can lead to choking.  Hoarseness.  Breath-holding and problems breathing during sleep.  Below-average intelligence.
NEUROLOGICAL COMPLICATIONS:  Many individuals with spina bifida have an associated abnormality of the cerebellum, called the Arnold Chiari II malformation. In affected individuals, the back portion of the brain is displaced from the back of the skull down into the upper neck.
EXECUTIVE FUNCTION:  Specific areas of difficulty in some individuals include planning, organizing, initiating, and working memory. Problem-solving, abstraction, and visual planning may also be impaired.  Children with spina bifida and shunted hydrocephalus have higher rates of ADHD.
ACADEMIC SKILLS:  Individuals with spina bifida may struggle academically, especially in the subjects of mathematics and reading. In one study, 60% of children with spina bifida were diagnosed with a learning disability.
SOCIAL COMPLICATIONS:  Compared to typically developing children, youths with spina bifida may have fewer friends and spend less time with peers.
DIAGNOSTIC EVALUATION: Pregnancy screening:  Neural tube defects can usually be detected during pregnancy by testing the mother's blood (AFP screening) or a detailed fetal ultrasound.  Increased levels of maternal serum alpha- fetoprotein (MSAFP) should be followed up by two tests - an ultrasound of the fetal spine and amniocentesis of the mother's amniotic fluid (to test for alpha-fetoprotein and acetylcholinesterase).
DIAGNOSTIC EVALUATION CONTD.. • Spina bifida occulta. Typically there aren't any signs or symptoms because the spinal nerves aren't involved. But you can sometimes see signs on the newborn's skin above the spinal defect, including an abnormal tuft of hair, or a small dimple or birthmark. Sometimes, the skin marks can be signs of an underlying spinal cord issue that can be discovered with MRI or spinal ultrasound in a newborn.
PREVENTION:  Dietary supplementation with folic acid has been shown to be helpful in reducing the incidence of spina bifida. Sources of folic acid include whole grains, fortified breakfast cereals, dried beans, leaf vegetables and fruits.  It is recommended that any woman considering becoming pregnant take 0.4 mg of folic acid a day. Pregnant women need 1 mg per day.
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 chest wall. abdomen or
CONTD…  Monitor growth and development of bones, muscles, and joints.  Treat and evaluate nervous system issues, such as seizure disorders.  Physical therapy  Speech therapy
IMMEDIATE CARE:  Place the child in prone position.  Cover the affected area with sterile gauze piece dipped in normal saline.  Maintain hydration.  Monitor for associated defects.
LIFE LONG TREATMENT:  Catheters  Braces  High fiber diet  Antibiotics may be used to treat or prevent infections such as meningitis or urinary tract infections.
COMPLICATIONS:  Difficult delivery with problems resulting from a traumatic birth, including cerebral palsy and decreased oxygen to the brain  Frequent urinary tract infections  Hydrocephalus  Loss of bowel or bladder control  Meningitis  Permanent weakness or paralysis of legs
COMPLICATIONS CONTD… • Possible problems in orthopedic issues such as: • Curved spine (scoliosis) • Abnormal growth • Dislocation of the hip • Bone and joint deformities • Muscle contractures • Shunt malfunction. Shunts placed in the brain to treat hydrocephalus can stop working or become infected. Warning signs may vary. Some of the warning signs of a shunt that isn't working include: • Headaches • Vomiting • Sleepiness • Irritability • Swelling or redness along the shunt • Confusion • Changes in the eyes (fixed downward gaze) • Trouble feeding • Seizures
COMPLICATIONS CONTD… • Infection in the tissues surrounding the brain (meningitis). • Tethered spinal cord. Tethered spinal cord results when the spinal nerves bind to the scar where the defect was closed surgically. The spinal cord is less able to grow as the child grows. This progressive tethering can cause loss of muscle function to the legs, bowel or bladder. Surgery can limit the degree of disability. • Sleep-disordered breathing. Both children and adults with spina bifida, particularly myelomeningocele, may have sleep apnea or other sleep disorders. • Skin problems. Children with spina bifida may get wounds on their feet, legs, buttocks or back. They can't feel when they get a blister or sore. Sores or blisters can turn into deep wounds or foot infections that are hard to treat. • Latex allergy. Children with spina bifida have a higher risk of latex allergy, an allergic reaction to natural rubber or latex products. Latex allergy may cause rash, sneezing, itching, watery eyes and a runny nose. It can also cause anaphylaxis
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Spina bifida

  • 2. DEFINITION:  Spina bifida (Latin: "split spine") is a developmental congenital disorder caused by the incomplete closing of the embryonic neural tube.  Some vertebrae overlying the spinal cord are not fully formed and remain unfused and open.  If the opening is large enough, this allows a portion of the spinal cord to protrude through the opening in the bones.  There may or may not be a fluid-filled sac surrounding the spinal cord.
  • 3. INCIDENCE:  Spina bifida is one of the most common birth defects, with an average worldwide incidence of one to two cases per 1000 births, but certain populations have a significantly greater risk.  Myelomeningocele is the most significant and common form, and this leads to disability in most affected individuals.  This condition is more likely to appear in females; the cause for this is unknown.
  • 4. RISK FACTORS • Race: morecommonamongHispanicsand whitesof Europeandescent. • Family history of neural tube defects. • However,mostbabieswithspinabifidaareborn toparentswithno knownfamilyhistoryofthe condition. • Folic acid deficiency (vitamin B-9) • Increased body temperature. Someevidence suggests that increased body temperature (hyperthermia)inthe early months ofpregnancy may increasetheriskofspinabifida.
  • 5. CAUSES:  Maternal diabetes  Family history  Obesity  Increased body temperature from fever or external sources such as hot tubs and electric blankets may increase the chances of delivery of a baby with a spina bifida.  Medications such as some anticonvulsants.  Pregnant women taking Valproic acid have an increased risk of having children with spina bifida  Genetic basis.  Folic acid deficiency
  • 6. EMBRYOLOGY:  Spina bifida is caused by the failure of the neural tube to close during the first month of embryonic development (often before the mother knows she is pregnant).  Under normal circumstances, the closure of the neural tube occurs around the 23rd (rostral closure) and 27th (caudal closure) day after fertilization.
  • 8. TYPES:  Spina bifida malformations fall into three categories:  spina bifida occulta  spina bifida cystica with meningocele  spina bifida cystica with myelomeningocele. (The most common location of the malformations is the lumbar and sacral areas)
  • 10. SPINA BIFIDA OCCULTA:  Occulta is Latin for "hidden". This is the mildest form of spina bifida.  In occulta, the outer part of some of the vertebrae is not completely closed.  The splits in the vertebrae are so small that the spinal cord does not protrude.  The skin at the site of the lesion may be normal, or it may have some hair growing from it; there may be a dimple in the skin, or a birthmark.  The incidence of spina bifida occulta is approximately 10% of the population, and most people are diagnosed incidentally from spinal X- rays
  • 11. MENINGOCELE:  The least common form of spina bifida is a posterior meningocele (or meningeal cyst). In this form, the vertebrae develop normally, but the meninges (pia & arachnoid) are forced into the gaps between the vertebrae. • Spinal cord is not involved. • There is no paralysis. • Sac contains only CSF • Surgical closure is done to prevent rupture and meningitis.
  • 13. MYELOMENINGOCELE:  This type of spina bifida often results in the most severe complications.  In individuals with myelomeningocele, the unfused portion of the spinal column allows the spinal cord to protrude through an opening.  The meningeal membranes that cover the spinal cord form a sac enclosing the spinal elements.  Spina bifida with myeloschisis is the most severe form of myelomeningocele. In this type, the involved area is represented by a flattened, plate-like mass of nervous tissue with no overlying membrane.  The exposure of these nerves and tissues make the baby more prone to life-threatening infections such as meningitis.
  • 15. CONTD…  The protruded portion of the spinal cord and the nerves that originate at that level of the cord are damaged or not properly developed.  As a result, there is usually some degree of paralysis and loss of sensation below the level of the spinal cord defect.
  • 16. CLINICALMANIFESTATIONS: Physical Signs:  Orthopedic abnormalities (i.e., club foot, hip dislocation)  Bladder and bowel control problems, including incontinence, urinary tract infections, and poor renal function.  Pressure sores and skin irritations  Abnormal eye movement  68% of children with spina bifida have an allergy to latex  Paralysis
  • 17. CONTD…  Scoliosis  Back pain  Partial or complete lack of sensation  Weakness of the hips, legs, or feet of a newborn  Other symptoms may include:  Hair at the back part of the pelvis called the sacral area  Dimpling of the sacral area  Difficulty swallowing, which can lead to choking.  Hoarseness.  Breath-holding and problems breathing during sleep.  Below-average intelligence.
  • 18. NEUROLOGICAL COMPLICATIONS:  Many individuals with spina bifida have an associated abnormality of the cerebellum, called the Arnold Chiari II malformation. In affected individuals, the back portion of the brain is displaced from the back of the skull down into the upper neck.
  • 19. EXECUTIVE FUNCTION:  Specific areas of difficulty in some individuals include planning, organizing, initiating, and working memory. Problem-solving, abstraction, and visual planning may also be impaired.  Children with spina bifida and shunted hydrocephalus have higher rates of ADHD.
  • 20. ACADEMIC SKILLS:  Individuals with spina bifida may struggle academically, especially in the subjects of mathematics and reading. In one study, 60% of children with spina bifida were diagnosed with a learning disability.
  • 21. SOCIAL COMPLICATIONS:  Compared to typically developing children, youths with spina bifida may have fewer friends and spend less time with peers.
  • 22. DIAGNOSTIC EVALUATION: Pregnancy screening:  Neural tube defects can usually be detected during pregnancy by testing the mother's blood (AFP screening) or a detailed fetal ultrasound.  Increased levels of maternal serum alpha- fetoprotein (MSAFP) should be followed up by two tests - an ultrasound of the fetal spine and amniocentesis of the mother's amniotic fluid (to test for alpha-fetoprotein and acetylcholinesterase).
  • 23. DIAGNOSTIC EVALUATION CONTD.. • Spina bifida occulta. Typically there aren't any signs or symptoms because the spinal nerves aren't involved. But you can sometimes see signs on the newborn's skin above the spinal defect, including an abnormal tuft of hair, or a small dimple or birthmark. Sometimes, the skin marks can be signs of an underlying spinal cord issue that can be discovered with MRI or spinal ultrasound in a newborn.
  • 24. PREVENTION:  Dietary supplementation with folic acid has been shown to be helpful in reducing the incidence of spina bifida. Sources of folic acid include whole grains, fortified breakfast cereals, dried beans, leaf vegetables and fruits.  It is recommended that any woman considering becoming pregnant take 0.4 mg of folic acid a day. Pregnant women need 1 mg per day.
  • 25. 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 chest wall. abdomen or
  • 26. CONTD…  Monitor growth and development of bones, muscles, and joints.  Treat and evaluate nervous system issues, such as seizure disorders.  Physical therapy  Speech therapy
  • 27. IMMEDIATE CARE:  Place the child in prone position.  Cover the affected area with sterile gauze piece dipped in normal saline.  Maintain hydration.  Monitor for associated defects.
  • 28. LIFE LONG TREATMENT:  Catheters  Braces  High fiber diet  Antibiotics may be used to treat or prevent infections such as meningitis or urinary tract infections.
  • 29. COMPLICATIONS:  Difficult delivery with problems resulting from a traumatic birth, including cerebral palsy and decreased oxygen to the brain  Frequent urinary tract infections  Hydrocephalus  Loss of bowel or bladder control  Meningitis  Permanent weakness or paralysis of legs
  • 30. COMPLICATIONS CONTD… • Possible problems in orthopedic issues such as: • Curved spine (scoliosis) • Abnormal growth • Dislocation of the hip • Bone and joint deformities • Muscle contractures • Shunt malfunction. Shunts placed in the brain to treat hydrocephalus can stop working or become infected. Warning signs may vary. Some of the warning signs of a shunt that isn't working include: • Headaches • Vomiting • Sleepiness • Irritability • Swelling or redness along the shunt • Confusion • Changes in the eyes (fixed downward gaze) • Trouble feeding • Seizures
  • 31. COMPLICATIONS CONTD… • Infection in the tissues surrounding the brain (meningitis). • Tethered spinal cord. Tethered spinal cord results when the spinal nerves bind to the scar where the defect was closed surgically. The spinal cord is less able to grow as the child grows. This progressive tethering can cause loss of muscle function to the legs, bowel or bladder. Surgery can limit the degree of disability. • Sleep-disordered breathing. Both children and adults with spina bifida, particularly myelomeningocele, may have sleep apnea or other sleep disorders. • Skin problems. Children with spina bifida may get wounds on their feet, legs, buttocks or back. They can't feel when they get a blister or sore. Sores or blisters can turn into deep wounds or foot infections that are hard to treat. • Latex allergy. Children with spina bifida have a higher risk of latex allergy, an allergic reaction to natural rubber or latex products. Latex allergy may cause rash, sneezing, itching, watery eyes and a runny nose. It can also cause anaphylaxis