general concepts of neural tube defects in pediatrics

1. NEURAL TUBE DEFECTS C. Ramos Perea, MD. FAAP. Associate Professor Universidad Central del Caribe y Escuela Medicina Ponce

3. Vertebral anatomy Vertebral arches(lamina ) Spinous process Vertebral canal Vértebra body TRANSVERSE FORAMEN Articular process Contains arteries & veins

7. Events in the 3-4 week

13. Anencephaly

24. Normal Folate Adequate Folate – Healthy Cells Deficient Folate – Unhealthy Cells Perspective on Folate Deficiency Megaloblastic Anemia

25. 50% of U.S. pregnancies are unplanned Folic acid is needed before conception NTDs occur very early in pregnancy Importance of timing in getting folic acid

26. Median serum and red blood cell folate levels, before and after folic acid fortification, U.S. women, aged 15-44 years, NHANES SOURCE: CDC/NCHS, National Health and Nutrition Examination Surveys, 1988-94 and 1999-2000 4.8 0 5 10 15 20 ng/mL Serum folate 13.0 Before After ng/mL 159.9 263.6 0 50 100 150 200 250 300 350 Red blood cell folate Before After

30. Spina Bifida Occulta

33. Meningomyelocele

37. Large occipital encephalocele

38. Lypomyelomeningocele The left child is a dorsal lipoma – pedunculated. The right child lypomatous mass beneath the skin. Both passed through the dura to the intradural space.

39. Spinal cord (close up) location of spina bifida Occulta Outer part of vertebrae not completely joined. Spinal cord and covering (meninges) usually undamaged. Hair often at site of defect. Meningocele Outer part of vertebrae split. Spinal cord usually normal. Meninges damaged and displaced through opening. Myelomeningocele Outer part of vertebrae split. Spinal cord and meninges damaged and displaced through opening. Usually hydrocephalus.

40. Anomalies Associated with Myelomeningocele Approximate Percent of Patients Chiari II malformation 90%+ Hydrocephalus 90%+ Syringomyelia 88% Brainstem malformations (cranial nerve) 75% Cerebral ventricle abnormalities 90%+ Cerebellar heterotopias 40% Cerebral heterotopias 40% Agenesis of the corpus callosum 12% Polymicrogyria 15-30%

43. Spina Bifida Occulta The child has long hairy tail with underline spina bifida occulta.

48. HAND OF HOPE

50. Hydranencephaly

51. Sirenomelia-mermaid

52. Onphalocele

53. Exencephaly

54. Gastroschisis

55. Encephalocele; Rachischisis; Iniencephaly

56. Holoprocencephaly sequence

57. Mid line anomalies “ El bosque seria muy triste si solo cantaran los pájaros que lo hacen mejor”rabindranath tagore