Spina bifida

NEURAL TUBE DEFECTS

1. NEURAL TUBE DEFECTS PREPARED BY:- Mrs.SHALI MSc NURSING MCON

2. INTRODUCTION During pregnancy, the brain and spine begin as a flat plate of cells. This plate rolls into a tube, called the neural tube. The tube is completely formed 28 to 32 days after conception. If all or part of the neural tube fails to close, this leaves an opening. The opening is called an open neural tube defect. The opening in the tube may be left exposed or covered with bone or skin

3. INTRODUCTION Neural tube defects are the congenital malformations of the CNS resulting from a defective closure of the neural tube during early embryogenesis between 3 rd and 4 th week of intrauterine life. It involves the defects in the skull, vertebral column, the spinal cord and other portion of CNS.

4. DEFINITIONS: The failure of closure of a portion of the neural tube disrupts the differnation of central nervous system induction of the vertebral arches. A number of developmental malformations of along the neuraxis from developing brain the sacrum that are called Neural Tube Defects

5. INCIDENCE It occurs in about 1 to 5 per 1000 live births.

6. ETIOLOGY Maternal radiation exposure, drugs( valproic acid), Exposure to chemicals, Malnutrition especially folic acid deficiency and Genetic factors

7. TYPES OF NEURAL TUBE DEFECTS

8. SPINA BIFIDA CYSTICA

9. Spina Bifida Spina Bifida latin word for split spine . Most common group of birth defects called neural tube defects (NTD ). The neural tube develops into the brain and spinal cord . B irth defect resulting from the incomplete closure of the embryonic neural tube

10. “Split Spine caused by incomplete closure of the neural tube, usually in the lumbar or sacral region What is spina bifida?

11. What is Spina Bifida? It’s a defect of the spine in a developing fetus, it affects the brain , spinal cord , and it’s muscles surrounding it, and resulting in loss of movement and or sensation to the legs and feet as well as bowel movement and bladder dysfunction . An unfortunately common birth defect that affects about 1,300 babies each year-

12. When does Spina Bifida occur? Occurs between days 24 and 28 of gestation. This disorders arise from abnormalities occurring during three major concurrent migrations of cell groups during embryogenesis which include those of the neural tube, the notochord, and the mesenchymal tissue .

13. It plays an important role in inducing formation of the spinal cord and spinal column.

14. Etiology History of previous affected pregnancy Inadequate intake of folic acid Pre gestational diabetics Valpronic acid and carbazepmine SUSPECTED: Maternal hyperthermia LES Chlorination of disinfection by produces in drinking water.

15. TYPES OF THE SPINA BIFIDA Occulta Meningocele Myelomeningocele

16. Three forms of Spina Bifida Occulta -There is a small defect or gap in one or more vertebrae of the spine, most affected individuals have no problems . Mildest form N o symptoms Gap in one or more of the vertebra of the spine Dimple , hairy patch, dark spot or swelling over affected area Spinal cords and nerves usually normal no treatment needed 

17. MENINGOCELE It is hernia protrusion of the meninges through a midline defect in the posterior vertebral arch. It forms a fluctuating cystic swelling filled with CSF and covered by a thin transparent membrane or with skin.

18. The Meningocele sac should be protected from infection and injury. The infant should be positioned on the abdomen to avoid pressure on the sac. If the sac is not covered with skin, it should be protected with sterile moist dressing.

19. Myelomeningocele Most severe form Cyst holds both membranes and nerve roots of spinal cord and, often, the cord itself Sometimes , a fully exposed section of spinal cord and nerves Closed surgically after birth affected babies: leg paralysis and bladder and bowel control problems 

20. Causes Causes of spina bifida are not completely understood scientists believe both genetic and environmental factors cause this However, 95% of affected babies are born to parents with no family history of these disorders 

21. CAUSES:- Women with certain chronic health problems such as diabetes and seizure disorders have an increased risk of having a baby with spina bifida especially if taking anticonvulsant medications. Approximately 1/100 of having a baby with spina bifida.

22. Who is at risk ? ANYONE !   Couples who already had an affected baby has an increased risk of having another affected baby.   Women who are obese, have poorly controlled diabetes, or who are treated with certain anti-seizure medications have a higher risk

23. Signs and symptoms :- Flaccid paralysis, absence of sensation and drop reflex. Postural abnormalities like club foot, subluxation of hips may be present. Spasticity and hyperactive Hydrocephalus is usually associated with this anomaly. contractures of joints, scoliosis and kyphosis may develop.

24. Screening and Diagnosis Prenatal Tests AFP Ultrasound Testing of Amniotic fluid Evaluation Analysis of individual medial history Physical examination Evaluation of critical body systems Imaging Studies X-rays Ultrasound CT scan MRI

25. What are the effects of Spina Bifida? Ongoing medical challenges Full or partial paralysis Nervous system complications Bladder and bowel control difficulties Learning disabilities Depression Social and sexual issues

26. Treatment No cure Regular check ups with physician Surgery (24 hours after birth) Medication Physiotherapy

27. How can spina bifida be managed? Detection Triple screening Maternal blood test for a-fetoprotein Ultrasound for bone defects Amniocentesis a-fetoprotein is elevated in 75-80% of cases of spina bifida (myelomeningocele)

28. How can spina bifida be managed ? Antibiotics In some cases the spinal cord is exposed to the environment Antibiotics are essential in preventing infection of the CNS

29. How Is Spina Bifida treated? One way that Spina Bifida is being treated is by operating on the fetus while still in the womb. This procedure is done as if fetus is being delivered via cesarean section . Another way is that it is usually treated surgically between 24 to48 hours after birth.

30. How can spina bifida be managed? Treatment for spina bifida depends on the extent of spinal cord involvement:- Spina bifida occulta usually requires no treatment unless pain from tethering develops Meningocele usually requires removal and early management of the cyst Myelomeningocele usually requires the most extensive treatment, but treatment is variable

31. Surgery Usually performed with in 24 hours after birth . They remove the infected area and replace it with muscle tissue and skin . Helps protect against hydrocephalus.

32. Assistive Devices Braces Crutches Wheel chairs

33. 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.

34. 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

35. Children with myelomeningocele often have hydrocephalus (blockage of CSF) Children may present with paralysis, blindness, MR, inability to speak, convulsions Any changes in mental status or behavior should be quickly brought to the attention of the child’s physician(s) Careful Observation

36. Goals of Intervention Teaching gross motor and spatial skills to facilitate coordination. Developing and maintaining fitness and flexibility to emphasize self sufficiency. Encouraging movement and fitness to overcome susceptibility to obesity. Developing physical skills that generalize to activities of daily living and vocational skills. Promoting social development in recreational and group play activities, such as aquatics. Providing appropriate social outlets for enjoyment and self satisfaction on an individual basis as well as with family or peers within the community .

37. Activities and Exercise Body Awareness Group Play Parachute activities Upper body activities Strength training Aquatics Flexibility Relays Wheel chair activities

38. Prevention Folic Acid -- 70% can be prevented multivitamin of 400 micrograms of folic acid every day  every day foods: grain products, fortified foods, leafy-green vegetables, dried beans, oranges, orange juice

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

40. DIAGNOSIS: - Primary diagnosis is done by clinical manifestations. Associated anomalies can be detected by X- ray, CT scan, MRI and complete neurological assessment. Routine blood and urine examination are also necessary. Prenatal diagnosis done by amniocentesis and estimation of alpha fetoprotein.

41. NURSING MANAGEMENT

42. Prevention of injury and infection of the sac by appropriate positioning with protective covering (sterile dressing) Prevention of skin break down and monitoring of signs of hydrocephalus( head circumference, fontanel, feeding behavior). Provision of adequate nutrition, promotion of urinary elimination and bowel regularity, prevention of leg or hip deformities and other complications.

43. Preoperative care, promotion of growth and development, emotional and psychological support, health teaching regarding care of the child are also important aspect of management.

44. Thank you for listening !