Spina bifida

Central nervous system defects include disorders caused by an imbalance of cerebrospinal fluid (as in hydrocephalus) and a range of disorders resulting from malformations of the neural tube during embryonic development (often called “neural tube defects”). These defects vary from mild to severely disabling.
Spina bifida is a birth defect where there is an incomplete closing of the backbone and membranes around the spinal cord. It is a developmental congenital anomaly

1. SPINA BIFIDA Presented by :- HARSHITA M.Sc. in Pediatric Nursing

2. SPINA BIFIDA SPINA BIFIDA IS A TYPE OF NEURAL TUBE DEFECT Spina bifida is a birth defect where there is incomplete closing of the backbone and membranes around the spinal cord.  It is a developmental congenital anomaly

3. PATHOPHYSIOLOGY Ectoderm outer most layer of fertilized egg develop a ridge, that eventually become neural tube. This neural tube form Spinal cord, Brain, Meninges Spina bifida occur when a portion of neural tube fail to close properly . This lead to defect or absence of vertebral arches to do failure of mesoderm to organize the defect.

4. CAUSES Specific is unknown. Multiple factors such as heredity and environment are thought to interact to produce these defects. The following have been identified as causative factors: low levels of maternal vitamins (B 9), including folic acid; obesity uncontrollable diabetes medication that interfere with folate metabolism hyperthermia during pregnancy.

5. TYPES SPINA BIFIDA Spina Bifida Occulta Meningocele Myelomeningocele

6. SPINA BIFIDA OCCULTA

7. SPINA BIFIDA OCCULTA Most common and least severe No protrusion of meninges Generally they are asymptomatic At most some children present with cutaneous lesion over the defect, as tufts of hair, dimple in the skin a birthmark or a mole on the skin ( naevus ), a benign tumour of fatty tissue (lipoma).

8. SPINA BIFIDA OCCULTA The symptomatic children usually present after 6-8 years of age with any of the following: - Progressive deformity of foot Change in micturition pattern Alteration in gait Trophic ulcers on the toes and feet.

9. SPINA BIFIDA OCCULTA Progressive disorder needs surgical correction. Laminectomy is performed for the condition and intraspinal lesion is excised. Surgery can be performed even before deficit appears, Myelogram CT scan and MRI helps to confirm the diagnosis

10. MENINGOCELE

11. MENINGOCELE It is a protrusion that includes the meninges and a sac containing cerebrospinal fluid (CSF). it is covered by normal skin. the spinal cord is not involved. It is generally found in lumbosacral region. It may also be found in the thoracic region and in skull.

12. MENINGOCELE The symptoms include Development of hydrocephalous- characterized by macrocephaly, headache, vomiting, urinary incontinence Spastic weakness of all four limbs An abnormally small sized head Uncoordinated muscle movements Delayed developmental milestones Vision problems Seizures 

13. MENINGOCELE Head circumference should be measured daily. Meningocele should be protected as risk of infection is present due to leakage of CSF. The child should be placed on abdomen to avoid pressure on the sac. Sac should be covered using sterile dressing. X Ray spine and skull CT scan should be done to rule out associated anomalies. Surgical closure of sac should be done as early as possible

14. MYELOMENINGOCELE

15. MYELOMENINGOCELE cystic sac of meninges with spinal tissue and CSF, which herniate through a defect in the posterior vertebral arch. They are of two types: Myelocele -open type Myelomeningocele – closed type

16. MYELOMENINGOCELE The child may present with Flaccid paralysis Absence of sensation Drop reflex Postural abnormalities (like club foot) Hydrocephalus is usually associated Musculoskeletal deformity Contracture of joints, Scliosis and kyphosis Risk of infection in CNS and rupture of sac

17. MYELOMENINGOCELE Chiari malformation type II. Chiari malformation ( kee -AH- ree mal-for-MAY-shun) type II is a common brain abnormality in children with the myelomeningocele form of spina bifida. The brainstem, or lowest part of the brain above the spinal cord, is elongated and positioned lower than usual. This can cause problems with breathing and swallowing. Rarely, compression on this area of the brain occurs and surgery is needed to relieve the pressure.

18. MYELOMENINGOCELE Primary diagnosis is done by clinical manifestations. X-Ray , CT Scan, MRI, and complete neurological assessment. Routine blood and urine examination Prenatal diagnosis can be done by amniocentesis and estimation of alpha fetoprotein. Management of this condition can be done by surgical correction of the defect. Correction of musculoskeletal deformities and regulation of bowel and bladder function. Additional supportive measure includes prevention of injury and infection of the sac by appropriate positioning with sterile dressing. Monitor head circumference Adequate nutrition

19. SPINA BIFIDA SPINA BIFIDA OCCULTA MENINGOCELE MYELOMENINGOCELE Most common and least severe Least common and not very severe Most severe No protrusions of tissue and spinal cord Only meninges are involved not the spinal nerve Spinal cord and surrounding meninges are involved At most people have tuft of hair, dimple or birth mark Protrusion that includes the meninges and a sac containing (CSF) Spinal cord and meninges protrude out of the vertebrae held together by sac of skin Usually no symptms Hydrocephalus, Spastic weakness small sized head, Seizures Uncoordinated muscle movements Delayed developmental milestones Vision problems, Seizures Loss of sensation, paralysis, bladder or bowel problem ,seizures, leg deformities

20. LABORATORY AND DIAGNOSTIC TESTS X-Rays- spine and skull MRI-spine and skull Prenatally it can be diagnosed by following screening tests, though these are non-specific: Serum Alpha Fetoprotein levels increases Ultrasonography Amniocentesis Blood test

21. MANAGEMENT (ASSESSMENT) Musculoskeletal Assessment and Neurologic Assessment Assess parents interactions with their infant and ability to cope with their child’s condition. Assess extent of motor and sensory involvement, and presence of reflexes. Assess for signs and symptoms of dehydration or fluid overload. Assess parents need for preoperative and postoperative information and support Assess for wound drainage and signs of infection. Assess for increased intracranial pressure. Assess parents and child’s ability to manage home treatment regimen. Assess parents’ and child’s needs for community services

22. DIAGNOSIS Risk for dysfunctional Grieving Interrupted Family processes Risk for Caregiver role strain, Impaired physical Mobility, Imbalanced Nutrition: less than body requirements Risk for Infection, Impaired Urinary elimination Bowel incontinence Impaired Skin integrity Knowledge Deficient Disturbed Body image Delayed Growth and development,

23. INTERVENTIONS(PRE-OPERATIVE) 1. Encourage parental expression of grief over loss of “perfect” child. a. Feelings related to guilt, self-blame b. Feelings of anger about child’s condition c. Feelings of inadequacy for procreating infant d. Feelings of being overwhelmed with the situation and the unknown 2. Provide emotional support to parents 3. Monitor infant’s vital signs and neurologic status.

24. INTERVENTIONS(PRE-OPERATIVE) 4. Promote optimal preoperative hydration and nutritional status. .5. Maintain integrity of defect; prevent further injury. 6. Prepare parents and infant for surgery

25. INTERVENTIONS(POST-OPERATIVE) Maintain nutritional and fluid intake Monitor for signs and symptoms of infections. Promote healing of surgical site; use sterile technique when changing and reinforcing dressing. Monitor vital signs and neurologic status. 5. Provide emotional support to parents.

26. DISCHARGE PLANNING AND HOME CARE Instruct parents about long-term management of bowel and bladder training. Provide information to parent and child about techniques to facilitate mobility and independence. Instruct parents on importance of child’s avoiding contact with latex or natural rubber. . Provide information about skin care and injury prevention. Provide education to parents about normal growth and development and deviations from norm.