HEHI Test #3

1. 44 inches long from medulla to lumbar vertebrae.
2. Nerves
3. Reflex
4. Protected by a sheath of bone and meninges
5. Surounded by pia mater (internal meninges), CSF, arachnoid, and dura mater (external meninges).

Many congenital defects happen before the woman even knows she's pregnant.

It closes between 15-29 days post conception. Failure to close normally results in neural tube defects.

90% of NTD are either spina bifida and anencephaly.

The occurrence of NTD is 1 in 5,000 births.

Both cerebral hemispheres are absent.

This NTD is incompatible with life. They are born stillborn and usually die of respiratory failure. The nurse HAS to have a DNR order (Do not resuscitate)

SB is failure of bony spine to close.

It occurs most often in LUMBAR and SACRAL areas.

Vertebra - 33 bony segments of spinal column that surround spinal cord.

Failur of the vertebrae to close WITHOUT PROTRUSION of spinal cord or meninges

Cutaneous manifestions (skin dimpling, tufts of hair or port wine stains) may OR may not occur.

Neuromuscular disturbances are most often asymptomatic/of no consequence.

Not a protrusion!! Patients with this NTD may have bladder problems, weakness, etc.

Visible defect with SAC-like PROTRUSION!!

Two kinds:
1. Meningocele - rare, contains meninges and CSF only

2. Myelomeningeocele - contains meninges, CSF, spinal cord, and/or nerve roots. This type is the most common of SB Cystica. (90%)

It is NOT associated with a neurological defect.

*GREATEST RISK - previous pregnancies with NTD
*Next greatest - Folic acid deficiency
*Females at risk over males
*Caucasians at risk over African Americans
*Maternal diabetes
*Obesity
*Anticonvulsants
*Maternal heat exposure (NO HOT TUBS or BATHS!!!)

*AAP rec. daily intake - 0.4 mg

*If there has been a previous NTD, mother should increase folic acid dose to 4.0 mg one month before pregnancy through 1st trimester.

*In 1998, the FDA authorized the fortification cereal grains with folic acid

Prenatal detection:
1. Ultrasound (16-18 weeks gestation)
2. AFP (Alpha-fetoprotein)
- Women who have pregnancies with NTD's usually have elevated AFP levels.
3. Fetal MRI
4. Amnio (14-17 weeks)

We want access to neurosurgery during delivery so it needs to occur at a tertiary care facility.

If the mother has an elective pre-labor C-section, then the baby will have a much better result in motor functioning versus the function from an EMERGENCY C-section.

This type of SBC is the most common. The location and extent of defect determines the deficit (Example: L2-L5 defects allow the baby to still be able to walk with
crutches)

Dxegree of neuro impairment:
Usually results in loss of sensation and complete/partial paralysis

*The most frequent anomaly associated with SB

*Monitor for S&S

*It may happen after closure of the defect.

Bladder - decreased nerve supply to the bladder results in a full bladder all the time.

Anal sphinctor tone - lack of bowel control. At risk for rectal prolapse.

Othopaedic abnormalities -
*dislocated hips
*club feet

Spinal deformities -
*scoliosis
*kyphosis

Initial care -

*prevention of infection: monitor closely for S&S of infection (increase or decrease temp, irritability, lethargy, nuchal rigidity)

*neuro assessment -
observe for associated anomalies (an ongoing process: daily head measurements)

*early closure

*basic needs of an infant

* Put in radiant warmer
* Put in prone position to decrease tension on the sac
* Put a sterile, moist dressing over defect
* Inspect the sac closely
* Observe for S&S of hydrocephalus, which are the same as increase cranial pressue:

- bulging fontanels
- dilated scalp veins
- high-pitched cry
- restless
- BP goes up
- HR goes down
- RR goes down/irregular

Goal - optimum GU functioning and decreased risk of GU distress.

Bowel control - diet modification, regular toilet habits

Tactile stimulation

Latex allergy - very significant with SB

Multi-disciplinary approach

NORMAL intellect should be assumed in the absence of significant hydrocephalus

*ARF sensory deficit (general)

*ARF infection (skin)

*nutrition less than body requirement (GI)

*ARF urinary retention (GU)

*ARF injury (MS)

Increased accumulation of CSF within the ventricles of the brain

*congenital - result of a mal-development

*acquired - something happens post-delivery

Explain this definition to parents:
"CSF is a water cushion protecting the brain and spinal cord from physical impact"

Medical definition: produced by secretion of choroid plexus of lateral and 3rd ventricles, circulates through out ventricles, and then absorbed within subarachnoid spaces - not clear how.

Communicating - impaired ABSORPTION because there is too much CSF trying to be absored into the blood stream.

Non-communicating - impaired/obstructed FLOW of CSF through ventricle system

*bulging forehead
*dilated scalp veins
*head growth at abnormal rate
(plot on growth curve; 35 cm = normal growth)
*frontal bossing
*setting sun eyes (eyes rotate downward and sclera is visible)
*irritable
*lethargic
*poor feeder (this is the FIRST symptom of anything)
*change in LOC
*paradoxical crying (the baby is happy until it is picked up - opposite of expected response)
*high pitched cry (neuro cry)

*irritable
*lethargic
*confused
*apathetic
*increased BP
*decreased pulse
*decreased or irregular RR

*HA after awakening, improves with emesis or sitting up

*ataxia

1. Antenatal by fetal ultrasound at 14 weeks
2. Increasing head circumference
3. Associated neurological signs
4. CT and MRI (PRIMARY diagnostic tools)

1. Ventriculoperitoneal shunt
(VP shunt)
- most common shunt; allows room for infant growth

2. Ventriculoatrial shunt
(VA shunt)

- The shunt procedures provide primary drainage of CSF from ventricles to extracrainial compartment - usually peritoneum.

1. sepsis or wound infection (serious, great risk 1-2 months after placement)

2. malfunction
- usually mechanical obstruction or displaced because of growth (most common reason for revision of shunt)

*increased ICP
*fever
*wound/shunt inflammation
*abdominal pain

Initially, give IV antibiotics for 7-10 days.

If it persists, remove shunt and place an EVD (extra ventricular drainage system)

Frequent assessment for S&S

Frequent small feedings

Ventricular tap (an advanced practice skill that is a sterile procedure that relieves pressure)

*Don't want to mask irritability!!!!*

Observe for S&S of shunt infection and malfunction

Position flat on un-operated side

Inspect operative site frequently for leaks, redness, and edema

Skin care - watch for pressure sores

Family support - it's a frightening condition

Family/patient teaching

NPO - 24 hours after

50-60% mortality rate

survivors have a high incidence of subnormal intellectual capacity

90% survival if they have little or no irreversible brain damage

2/3 of the survivors are intellectually normal, assuming there aren't other issues

1. Bacterial (pyogenic)
2. Viral (aseptic)
3. Tuberculous

Neonates (group B strep, e coli, listeria)


Over 2 months of age (H flu, strep pneumoniae, nisseria meningitidis)

MOST COMMON ROUTE - vascular dissemination from a focus of infection elsewhere; most often an extension of a nasopharyngeal infection that enters the blood and goes to cerebral blood flow and enters the blood/brain barrier and infects it.

Direct entry of organisms from

*post LP or surgical procedures

*anatomic abnormalities (spina bifida)

*foreign body (VP shunt)

Infective process

*inflammation

*exudate

*WBC accumulation

*tissue damage

Brain

*hyperemic

*brain covered with purulent exudate

*can lead to obstructive exudate

1. *abrupt onset
*fever
*chills
*HA
*vomiting
*change in sensorium
*irritable
*agitated
*photophobia
*confusion
*hallucinations
*drowsiness
*stupor
*coma

2. Meningocococcal - purpuric/petechial rash

3. Nuchal rigidity

4. Kernig sign (positive) - assess pt. flat on back, bend and then straighten out the leg and they will feel pain in legs if it is positive.

5. Brudzinski sign (positive)-
assess pt. flat on back, flex neck and assess reflex contractions of hips and knees.

6. Seizures in a child without a seizure disorder is the first sign.

6. children ages 3 mo. to 2 yrs.
(bulging fontanel - MOST significant sign because it's a release for increased ICP; fever; poor feeding; vomit irritable; restless; high pitched cry "neuro cry")

7. Nuchal rigidity, Brudzinski, Kernig - not consistently present in young child less than 18 months

8. Neonates - difficult to diagnose, vague like all neonatal sepsis. They PRESENT with the sign of poor feeding. They also have poor suck, poor muscle tone, hypothermia/fever, resp irref, apnea, weight loss, irritable, drowsy, bulging fontanel (may or may not appear)

9. Neck is usually supple so you will not be looking for rigidity.

Know the trends!!

Lumbar puncture - definitive test

1. increased spinal fluid pressure

2. increased WBC

3. decreased glucose level

4. Increase protein concentration

5. cloudy in color (CSF should be clear)

6. positive gram stain - this is the GOLD STANDARD

**Highest morbidity and mortality occurs in children under four years old.**

*obstructive hydrocephalus

*deafness, blindness, paralysis of facial muscles

*CP (cerebral palsy)

*mental handicap

*learning disorder

*ADHD

*seizures

1. isolation

2. antimicrobial therapy

3. maintain hydration

4. ventilation

5. decrease increased ICP

6. manage systemic shock

7. control seizures

8. control temp

9. treat complications

1. Environment - quiet w/ decreased stimuli; no lights or noise.

2. Position - let them assume position of choice

3. Pain evaluation - check frequently and manage it

4. Safety - take seizure precautions

5. Frequent assessment

6. Frequent vital signs, neuro checks, LOC, I&O

7. Family support - openly discuss feelings with the family.

Diagnosis - CSF

WBC - slightly elevated

Protein - within normal limits or slight increase

Glucose - WNL

Color - clear/slightly cloudy, gram stain neg

Treatment - symptomatic

*hydration
*pain management
*positioning