HYDROCEPHALUS (1).ppt

8/11/2019 HYDROCEPHALUS (1).ppt

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HYDROCEPHALUS

W & W 495-503


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Hydrocephalus A syndrome, or sign, resulting fromdisturbances in the dynamics of

cerebrospinal fluid (CSF), which maybe caused by several diseases.


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Incidence Occurs in 3-4 of every 1000 births. Cause may be congenital or acquired.

Congenital- may be due tomaldevelopment or intrauterineinfection

Acquired- may be due to infection,neoplasm or hemorrhage.


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Pathophysiology CSF is formed by two mechanisms: Secretion by the choroid plexus,

Lymphatic-like drainage by theextracellular fluid in brain.

CSF circulates thru ventricular systemand is absorbed within subarachnoidspaces by unknown mechanism.


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Mechanisms of Fluid

Imbalance Hydrocephalus results from: 1. Impaired absorption of CSFwithin

the subarachnoid space(communicating hydrocephalus), or

2. Obstruction to the flow of CSF

through the ventricular system (non-communicating hydrocephalus)


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Mechanisms of fluid

imbalance Both lead to increase accumulation ofCSF in the ventricles!

Ventricles become dilated andcompress the brain. When this happens before cranial

sutures are closed, skull enlarges. In children


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Hydrocephalus Most cases of non-communicating(obstructive) hydrocephalus are a result ofdevelopmental malformations.

Other causes: neoplasms, intrauterineinfections, trauma. Developmental defects account for most

causes of hydrocephalus from birth to 2years of age. (Table 11-3, page 497- sitesand types of hydrocephalus)


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Common Defects Arnold-Chiari Malformation (ACM) Type 2 malformation of brain seen most

exclusively with myelomeningocele, ischaracterized by herniation of a smallcerebellum, medulla, pons, and fourthventricle into the cervical spinal canal

through an enlarged foramen magnum.


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Clinical manifestations Clinical picture depends on acuity ofonset and presence of preexisting

structural lesions.


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Infancy Head grows at alarming rate withhydrocephalus.

First signs- bulging of fontanels withouthead enlargement.

Tense, bulging, non-pulsatile anteriorfontanel

Dilated scalp veins, esp. when crying

Thin skull bones with separated sutures(cracked pot sounds on percussion)


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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.


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Infancy Swallowing difficulties, stridor,apnea, aspiration, respiratory

difficulties and arm weakness mayindicate brain stem compression.

If hydrocephalus progresses,

difficulty sucking and feeding, and ahigh-pitched shrill cry results. (lowerbrain stem dysfunction)


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Infancy Emesis, somnolence, seizures, andcardiopulmonary distress ensues and

hydrocephalus progresses. Severely affected infants may not

survive neonatal period.


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Childhood Signs and symptoms caused byincreased ICP.

Manifestations caused by posteriorneoplasms and aqueduct stenosis,manifestations associated with

space-occupying lesions.


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Childhood

Headache on awakening with improvementfollowing emesis or sitting up.

Papilledema (swelling of optic disc DTobstruction), strabismus, andextrapyramidal tract signs such as ataxia

Irritability, lethargy, apathy, confusion,

and often incoherent


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Childhood Dandy-Walker syndrome- congenitaldefect-late onset.

Obstruction of foramen of Lushka andMagendie

Bulging occiput, nystagmus, ataxia,cranial nerve palsies

Female predominance (3:1)

Absence or occlusion of ventricles


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Diagnostic Evaluation Antenatal- fetal ultrasound as early as 14weeks

Infancy- based on head circumference

crosses one or more grid lines on theinfant growth chart within a 4 week periodand there are progressive neuro signs.

CT and MRI to localize site of obstruction;

reveal large ventricles


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Therapeutic management Goals: Relieve hydrocephaly

Treat complications Manage problem resulting from

effects of disorder on psychomotor

development USUALLY SURGICAL!


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Surgical Treatment Therapy of choice! Direct removal of source of

obstruction (neoplasm, cyst, orhematoma)

Most require shunt procedure to

drain CSF from ventricles toextracranial area; usuallyperitoneum(VP shunt), or right atrium

(VA shunt) for absorption.


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VP shunt Used in neonates and young infants Greater allowance for excess tubing;

which minimizes number of revisionsneeded as child grows


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VA shunt Reserved for older children who haveattained most of somatic growth, or

children with abdominal pathology. Contraindicated in children with

cardiopulmonary disease or with

elevated CSF protein.


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Major Complications Shunt infection is most seriouscomplication!

Period of greatest risk is 1 to 2months following placement.

Staph and strep most common

organisms


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Complications Mechanical difficultieskinking, plugging, migration of

tubing. Malfunction is most often by

mechanical obstruction!

Look for signs of increased ICP;fever, inflammation and abdominalpain.


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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 dueto small vein rupture in cerebral cortex.

3. Do not pump shunt without specificdirection from doctor (too manydifferent pump devices)


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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 aspressure. Blood pressure may be variable due to hypoxia

to brainstem Abdominal distention- due to CSF peritonitis or

post-op ileus due to catheter placement.


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Post-op 5. Monitor I and O- may be on fluidrestriction or NPO for 24 hours to

prevent fluid overload. 6. Monitor VS- increased temp mayindicate infection.

7. Give good skin care to preventtissue damage, etc.


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Family support Fear Communication of procedures

Prepare for discharge.


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SPINA BIFIDA Neural Tubedefects are largestgroup of congenital

anomalies. Failure of neural

tube to close

produces defectsof either entireneural tube orsmall areas.
http://www.cdc.gov/ncbddd/folicacid/excite/images/spina.gif


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Etiology Anacephaly and spina bifida occurtogether very often.

Higher in females than males 50% occur due to nutritional

deficiency (folic acid)


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Spina Bifida Defined as midline defects involving failure of thebony 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 otherneuromuscular manifestations.

Many people with occulta will never have any deficits andmay not know they have it.


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Spina Bifida Spina Bifida cystica- visible defectwith external saclike protrusion.

A. meningocele- encases meninges andspinal fluid, but no neurological deficits.

B. meningomyelocele-contains meninges,spinal fluid, and nerves. Neuromotor

deficits depend on anatomic level ofprotrusion and nerves involved.


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http://images.google.com/imgres?imgurl=www.spinabifidainfo.nl/spbifida_baby1.jpg&imgrefurl=http://www.spinabifidainfo.nl/spina_bifida.htm&h=397&w=260&prev=/images%3Fq%3Dspina%2Bbifida%26svnum%3D10%26hl%3Den%26lr%3D%26ie%3DUTF-8%26oe%3DUTF-8


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Meningomyelocele AKA spina bifida Develops during first 28 days of

pregnancy when neural tube fails toclose 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.


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meningomyelocele Largest number in lumbar or lumbosacralarea

90-95% of children have hydrocephalus

Careful monitoring of head size important Chiari malformation may be present:

observe infant for stridor, hoarse cryfrom vocal cord paralysis; feedingdifficulties, deteriorating upper extremityfunction.


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Clinical manifestations S/S vary according to degree of spinaldefect.

Readily apparent on inspection!

Loss of sensation below lesion

Poor urinary and bladder control

Joint deformities in lower extremities

Scoliosis or kyphosis

Hip dislocations


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Diagnostic evaluation Examination of meningeal sac and clinicalmanifestations

MRI, CT to assess condition of brain and spinalcord. Other defects may be present.

Prenatal- fetal ultrasound or amniotic fluid samplefor (alpha-fetal protein(AFP).

Test should be done between 16 and 18 weeks ofgestation. Afterwards AFP level drops, making

detection of SB difficult. Also, therapeuticabortion not option after this time.


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Therapeutic management Multi-disciplinary approach Neurology, neuro-surgery, pediatrics,

urology, orthopedics, rehabilitation, PT,OT, social services, intensive nursing inmany areas.


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Goals 1. prevent infection 2. early closure of lesion, within 72

hours (prevents infection and traumato exposed tissues, and preventsfurther motor impairment). Goal issatisfactory skin coverage of lesion!

3. PT for specific deformity


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Goals Physical therapy to prevent jointcontractures.

Correct deformity, prevent skinbreakdown, obtain best ambulatoryfunctioning


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Goals Management of genitourinaryfunction: Mylemeningocele is a common cause of

neurogenic bladder which leads tourinary system distress (frequent UTIs,ureterohydronephrosis, vesicoureteralreflux, renal insufficiency.

Urinary incontinence is common


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Goals Clean, intermittent catherization as aconservative treatment.

Vesicostomy (anterior wall of bladderbrought through abdominal wall tocreate stoma) may be done forbladder control.

Meticulous skin care is needed!


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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, pressureareas a threat, feeding a problem


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Nursing care Pre-op Turn head on side for feeding

Diapering may be contraindicated untilrepair and healing has taken place.

Constant stooling due to affected bowelsphincter (not diarrhea).

Keep skin clean.


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Post-op care Prone position until healing takesplace!

May allow side-lying- depends ondoctor.

Feeding resumed after anesthesiawears off

Comfort measure/vital signs/I & O


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Latex allergy 70% of children and adolescents withSB are sensitive to latex.

Cause unknown-probably continuedexposure to latex.

Avoid latex, balloons, condoms, catheters,

or anything with latex!


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Post-op Use touch for stimulation, since canthold

Observe for increased ICP, such asbulging fontanels

Assess for infection: Increased or decreased temperature,

irritability, nuchal rigidity,


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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


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HYDROCEPHALUS (1).ppt

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