B.Sc Nursing 3 Year

1. Congenital Anomalies of Central
Nervous System

2. Learning Objectives
 Recognize Anomalies of Head
 Hydrocephalus,Microcephaly
 How do they Present clinically
 Recognize anomalies of Spine
 Neural Tube defects
 Spina bifida
 Recognize clinical manifestations

3. HYDROCEPHALUS
 Congenital
 Acquired
 Excessive accumulation of cerebrospinal fluid
(CSF) in the cerebral cavity .
 There is either impaired absorption or increased
production of CSF.
 There Is obstruction to the flow of CSF and
dilatation occurs distal to obstruction

5. Clinical features
 IN INFANTS
 Accelerated rate of enlargement of head
 Anterior fontanel is wide and bulging
 Scalp veins are dilated
 The forehead is broad
 Fixed downward gaze
 And sunsetting of eyes

10. OLDER CHILDREN
 Irritability,lethargy
 poor appetite
 vomiting,headache
 A gradual change in
personality and
intellectual
productivity
 Serial measurement in
head circumference
shows increased
velocity

11. EXAMINATION
Cracked Pot sign
 Papilledema,
 Abducens nerve palsy
 Pyramidal tract sign more evident in lower limbs

12. MACROCEPHELY
 Head circumference above 98th percentile
 Normal children may have large heads
 Familial
 Tall stature
 Hydrocephalus
 Subdural hematoma,Cerebral tumour,
 Cerebral gigantism

13. MICROCEPHALY
 Head circumference below 2nd centile
 Abnormalities in fetal development during
neuronal migration
 Familial
 Autosomal recessive condition with
developmental delay
 Congenital infection
 Perinatal Asphyxia,meningitis

16. NEURAL TUBE DEFECTS
 Failure of the neural tube to close spontaneously
between 3rd & 4th week in utero development
 CAUSES
 Maternal Diabetes
 Maternal obesity
 Mutations in folate dependent or folate
responsive pathways

17. MAJOR DEFECTS
 Spina bifida occulta
 Meningocele
 Myelomeningocele
 Encephalocele
 Anancephaly
 Dermal sinus
 Tethered cord

18. SPINA BIFIDA OCCULTA
 Midline defect of the vertebral bodies without
protrusion of the spinal cord or meninges
 Mostly asymptomatic
 In the midline of the lower back(L5 S1)
Patches of hair,
Lipoma,
Discolouration of skin
Dermal sinus
 ,

19. MENINGOCELE
 Meninges herniate through a defect in
posterior vetebral arches
 A fluctuating midline mass that transilluminates
occurs along the vetebral column in lower back
well covered with skin

23. MYELOMENINGOCELE
 Most severe form of dysraphism
 1 in 4000 live births
 Lumbosacral in 75% of cases.
 Dysfunction of many organs and
structures including skin, skeleton,
gastrointestinal and genitourinary tract.

25. NEUROLOGIC DEFICIT
 Depends on the location of defect
 Low sacral
 Bladder and Bowel incontinence
 Mid Lumber Region
 Flaccid paralysis of lower limbs
 Absent deep tendon reflexes
 Lack of response to touch and pain
 High incidence of lower extremity deformities.

26. ANENCEPHALY
 A defect in the calvarium
meninges and scalp
 Rudimentary brain
 failure of closure of rostral neuropore (opening of
the anterior neural tube.)
 The cerebral hemispheres and cerebellum are
absent and a residue of brainstem present

28. PREVENTION
 Folic Acid Tablets
 4 mg daily one month before conception