All about the hydrocephalus and spina bifida child

1. HYDROCEPHALUS
W & W 495-503

2. Hydrocephalus
• A syndrome, or sign, resulting from
disturbances in the dynamics of
cerebrospinal fluid (CSF), which may
be caused by several diseases.

4. Incidence
• Occurs in 3-4 of every 1000 births.
• Cause may be congenital or acquired.
• Congenital- may be due to
maldevelopment or intrauterine
infection
• Acquired- may be due to infection,
neoplasm or hemorrhage.

5. Pathophysiology
• CSF is formed by two mechanisms:
– Secretion by the choroid plexus,
– Lymphatic-like drainage by the
extracellular fluid in brain.
CSF circulates thru ventricular system
and is absorbed within subarachnoid
spaces by unknown mechanism.

7. Mechanisms of Fluid
Imbalance
• Hydrocephalus results from:
• 1. Impaired absorption of CSF within
the subarachnoid space
(communicating hydrocephalus), or
• 2. Obstruction to the flow of CSF
through the ventricular system (non-
communicating hydrocephalus)

8. Mechanisms of fluid
imbalance
• Both lead to increase accumulation of
CSF in the ventricles!
• Ventricles become dilated and
compress the brain.
• When this happens before cranial
sutures are closed, skull enlarges.
• In children <10-12, previously closed
sutures may open.

9. Hydrocephalus
• Most cases of non-communicating
(obstructive) hydrocephalus are a result of
developmental malformations.
• Other causes: neoplasms, intrauterine
infections, trauma.
• Developmental defects account for most
causes of hydrocephalus from birth to 2
years of age. (Table 11-3, page 497- sites
and types of hydrocephalus)

10. Common Defects
• Arnold-Chiari Malformation (ACM)
– Type 2 malformation of brain seen most
exclusively with myelomeningocele, is
characterized by herniation of a small
cerebellum, medulla, pons, and fourth
ventricle into the cervical spinal canal
through an enlarged foramen magnum.

12. Clinical manifestations
• Clinical picture depends on acuity of
onset and presence of preexisting
structural lesions.

13. Infancy
• Head grows at alarming rate with
hydrocephalus.
– First signs- bulging of fontanels without
head enlargement.
– Tense, bulging, non-pulsatile anterior
fontanel
– Dilated scalp veins, esp. when crying
– Thin skull bones with separated sutures
(cracked pot sounds on percussion)

17. Infancy
• Protruding forehead or bossing.
• Depressed eyes or setting-sun eyes (eyes
rotating or downward with sclera visible
above pupil)
• Pupils sluggish with unequal response to
light
• Irritability, lethargy, feeds poorly,
changes in LOC, arching of back
(opisthotonos), lower extremity spasticity.
• May cry when picked up or rocked; quiets
when allowed to lay still.

18. Infancy
• Swallowing difficulties, stridor,
apnea, aspiration, respiratory
difficulties and arm weakness may
indicate brain stem compression.
• If hydrocephalus progresses,
difficulty sucking and feeding, and a
high-pitched shrill cry results. (lower
brain stem dysfunction)

19. Infancy
• Emesis, somnolence, seizures, and
cardiopulmonary distress ensues and
hydrocephalus progresses.
• Severely affected infants may not
survive neonatal period.

21. Childhood
• Signs and symptoms caused by
increased ICP.
• Manifestations caused by posterior
neoplasms and aqueduct stenosis,
manifestations associated with
space-occupying lesions.

22. Childhood
• Headache on awakening with improvement
following emesis or sitting up.
• Papilledema (swelling of optic disc DT
obstruction), strabismus, and
extrapyramidal tract signs such as ataxia
• Irritability, lethargy, apathy, confusion,
and often incoherent

24. Childhood
• Dandy-Walker syndrome- congenital
defect-late onset.
– Obstruction of foramen of Lushka and
Magendie
– Bulging occiput, nystagmus, ataxia,
cranial nerve palsies
– Female predominance (3:1)
– Absence or occlusion of ventricles

25. Diagnostic Evaluation
• Antenatal- fetal ultrasound as early as 14
weeks
• Infancy- based on head circumference
crosses one or more grid lines on the
infant growth chart within a 4 week period
and there are progressive neuro signs.
• CT and MRI to localize site of obstruction;
reveal large ventricles

26. Therapeutic management
• Goals:
• Relieve hydrocephaly
• Treat complications
• Manage problem resulting from
effects of disorder on psychomotor
development
• USUALLY SURGICAL!

27. Surgical Treatment
• Therapy of choice!
• Direct removal of source of
obstruction (neoplasm, cyst, or
hematoma)
• Most require shunt procedure to
drain CSF from ventricles to
extracranial area; usually
peritoneum(VP shunt), or right atrium
(VA shunt) for absorption.

29. VP shunt
• Used in neonates and young infants
• Greater allowance for excess tubing;
which minimizes number of revisions
needed as child grows

31. VA shunt
• Reserved for older children who have
attained most of somatic growth, or
children with abdominal pathology.
• Contraindicated in children with
cardiopulmonary disease or with
elevated CSF protein.

34. Major Complications
• Shunt infection is most serious
complication!
• Period of greatest risk is 1 to 2
months following placement.
• Staph and strep most common
organisms

35. Complications
• Mechanical difficulties
kinking, plugging, migration of
tubing.
• Malfunction is most often by
mechanical obstruction!
• Look for signs of increased ICP;
fever, inflammation and abdominal
pain.

36. Post-op care
• In addition to routine post-op care:
– 1. Place on unoperated side to prevent
pressure on shunt valve
– 2. Keep HOB flat; rapid decrease in IC
fluid may cause subdural hematoma due
to small vein rupture in cerebral cortex.
– 3. Do not pump shunt without specific
direction from doctor (too many
different pump devices)

37. Post-op care
• 4. Observe for signs of Increased ICP!
May indicate obstruction of shunt!
– Assess pupil size; as pressure on oculomotor
nerve may cause dilation on same side as
pressure.
– Blood pressure may be variable due to hypoxia
to brainstem
– Abdominal distention- due to CSF peritonitis or
post-op ileus due to catheter placement.

38. Post-op
• 5. Monitor I and O- may be on fluid
restriction or NPO for 24 hours to
prevent fluid overload.
• 6. Monitor VS- increased temp may
indicate infection.
• 7. Give good skin care to prevent
tissue damage, etc.

39. Family support
• Fear
• Communication of procedures
• Prepare for discharge.

40. SPINA BIFIDA
• Neural Tube
defects are largest
group of congenital
anomalies.
• Failure of neural
tube to close
produces defects
of either entire
neural tube or
small areas.

41. Etiology
• Anacephaly and spina bifida occur
together very often.
• Higher in females than males
• 50% occur due to nutritional
deficiency (folic acid)

42. Spina Bifida
• Defined as midline defects involving failure of the
bony spine to close.
• Spina bifida occulta- defect not visible externally.
– Occurs most often in lumbosacral area.
– Not apparent unless there are gait disturbances, foot
deformities, sphincter dysfunction or other
neuromuscular manifestations.
– Many people with occulta will never have any deficits and
may not know they have it.

43. Spina Bifida
• Spina Bifida cystica- visible defect
with external saclike protrusion.
– A. meningocele- encases meninges and
spinal fluid, but no neurological deficits.
– B. meningomyelocele-contains meninges,
spinal fluid, and nerves. Neuromotor
deficits depend on anatomic level of
protrusion and nerves involved.

47. Meningomyelocele
• AKA spina bifida
• Develops during first 28 days of
pregnancy when neural tube fails to
close and fuse.
• 90% of spinal cord lesions, and may
occur at any point along spine.
• Sac usually enclosed in fine
membrane that is prone to tears.

48. meningomyelocele
• Largest number in lumbar or lumbosacral
area
• 90-95% of children have hydrocephalus
• Careful monitoring of head size important
• Chiari malformation may be present:
observe infant for stridor, hoarse cry
from vocal cord paralysis; feeding
difficulties, deteriorating upper extremity
function.

49. Clinical manifestations
• S/S vary according to degree of spinal
defect.
• Readily apparent on inspection!
• Loss of sensation below lesion
• Poor urinary and bladder control
• Joint deformities in lower extremities
• Scoliosis or kyphosis
• Hip dislocations

50. Diagnostic evaluation
• Examination of meningeal sac and clinical
manifestations
• MRI, CT to assess condition of brain and spinal
cord. Other defects may be present.
• Prenatal- fetal ultrasound or amniotic fluid sample
for (alpha-fetal protein (AFP).
• Test should be done between 16 and 18 weeks of
gestation. Afterwards AFP level drops, making
detection of SB difficult. Also, therapeutic
abortion not option after this time.

52. Therapeutic management
• Multi-disciplinary approach
– Neurology, neuro-surgery, pediatrics,
urology, orthopedics, rehabilitation, PT,
OT, social services, intensive nursing in
many areas.

53. Goals
• 1. prevent infection
• 2. early closure of lesion, within 72
hours (prevents infection and trauma
to exposed tissues, and prevents
further motor impairment). Goal is
satisfactory skin coverage of lesion!
• 3. PT for specific deformity

54. Goals
• Physical therapy to prevent joint
contractures.
– Correct deformity, prevent skin
breakdown, obtain best ambulatory
functioning

55. Goals
• Management of genitourinary
function:
– Mylemeningocele is a common cause of
neurogenic bladder which leads to
urinary system distress (frequent UTI’s,
ureterohydronephrosis, vesicoureteral
reflux, renal insufficiency.
– Urinary incontinence is common

56. Goals
• Clean, intermittent catherization as a
conservative treatment.
• Vesicostomy (anterior wall of bladder
brought through abdominal wall to
create stoma) may be done for
bladder control.
• Meticulous skin care is needed!

57. Nursing care
• Pre-op
– Positioning to keep off sac (use diaper
rolls, pads or sandbags)
– Keep in prone position
– Can be challenging!
– More difficult to keep clean, pressure
areas a threat, feeding a problem

58. Nursing care
• Pre-op
– Turn head on side for feeding
– Diapering may be contraindicated until
repair and healing has taken place.
– Constant stooling due to affected bowel
sphincter (not diarrhea).
– Keep skin clean.

59. Post-op care
• Prone position until healing takes
place!
• May allow side-lying- depends on
doctor.
• Feeding resumed after anesthesia
wears off
• Comfort measure/vital signs/I & O

60. Latex allergy
• 70% of children and adolescents with
SB are sensitive to latex.
– Cause unknown-probably continued
exposure to latex.
Avoid latex, balloons, condoms, catheters,
or anything with latex!

61. Post-op
• Use touch for stimulation, since can’t
hold
• Observe for increased ICP, such as
bulging fontanels
• Assess for infection:
– Increased or decreased temperature,
irritability, nuchal rigidity,

62. Home Care
• Involve parents in care
– Positioning/feeding/skin care/range of
motion exercises/ clean catheterization
when prescribed, complications.
– Help with assistive devices (if child is
paraplegic use hands/arms, etc.)
– Long range planning
– Spina Bifida Association of America