Mrs.AMANLO KAUR

To define hydrocephalus. To enlist the causes of hydrocephalus. To discuss the physiology of formation of

CSF production & flow. To explain types of hydrocephalus & their

pathophysiology. To differentiate the clinical manifestation in

infants & in childhood. To enlist diagnostic evaluation for

hydrocephalus. To describe the management of

hydrocephalus- medical, surgical & nursing

Term derived from two Greek words- “hydro” means water and “cephalus” means head.

This condition is sometimes known as “water in the brain”.

Hydrocephalus is a condition caused by an imbalance in the production and absorption of CSF in the ventricular system.

When production exceeds absorption, CSF accumulates, usually under pressure, producing dilation of the ventricles.

Hydrocephalus is a build up of fluid inside the skull, leading to brain swelling.

Incidence of primary hydrocephalus without spina bifida is approximately 1 in 2500 live births, making it one of the most common developmental disabilities, more common than Down syndrome or deafness. It is the leading cause of brain surgery for children.

In the past 25 years, death rates associated with hydrocephalus have decreased from 54% to 5% and the occurrence of intellectual disability has decreased from 62% to 30%.

1) CONGENITAL HYDROCEPHALUSI. Intrauterine infections: Rubella,

Cytomegalovirus, Toxoplasmosis.II. Trauma: Subarachnoid, Intracranial,

Intraventricular haemorrhages.III.Congenital malformations: Dandy-walker syndrome: here posterior

fossa cyst continuous with 4th ventricle.

Aqueduct stenosis: it accounts for 33% of hydrocephalus cases. Stenosis of aqueduct of sylvius causes dilation of lateral and 3rd ventricles. In 2% of cases this could be familial with X linked recessive inheritance.

Arnold-Chiari syndrome- Portions of cerebellum & brainstem herniating into cervical spinal canal, blocking the flow of CSF to the posterior fossa.

 

2) ACQUIRED HYDROCEPHALUS: - Tuberculosis, chronic & pyogenic

meningitis. Post-intraventricular hemorrhage. Posterior fossa tumors:

medulloblastoma, astrocytoma, ependymoma.

Arteriovenous malformation, intracranial hemorrhage, ruptured aneurysm.

CSF is secreted at the choroid plexus within the ventricles by ultra filtration & active secretion.

Lateral ventricles

FORAMEN OF MONRO

3rd ventricle

AQUEDUCT OF SYLVIUS

4th ventricle

CONTD…

Via lateral foramen of luschka & foramen of magendie

Cistern magna

Cerebral & cerebellar subarachnoid spaces

16

17

A large portion is absorbed through the arachnoid villi, but the sinuses, veins, brain substance & dura also participate in absorption.

About 20ml of CSF is secreted in an hour. The total of CSF approximates 50ml in an infant and 150ml in adults.

There are two types of hydrocephalus: -1) Noncommunicating(intraventricular

or obstructive) hydrocephalus-In this there is blockage between the ventricular & subarachnoid systems, resulting in an interference with circulation of CSF & lack of access to the subaracnoid spaces. In this fluid distends the ventricles. There is a gradual thinning of the brain substance, which is compressed between the distended ventricles & the expanding skull.

Noncommunicating hydrocephalus may be due to stenosis of the aqueduct of sylivus, either a congenital defect or acquired.

Obstructive hydrocephalus may result postnatally from brain tumors that put pressure on or extend into the ventricles or circulation pathways.

2) Communicating (extraventricular)hydrocephalus- In this there is normal communication between the ventricles & the spinal subarachnoid space. There is an interference with the absorption of CSF caused by an occlusion of the subarachnoid cisterns around the brain stem. The fluid that is not absorbed in the subarachnoid space accumulates, compressing the brain & distending the cranial cavity.

Communicating hydrocephalus may be due to subarachnoid hemorrhage or meningitis, toxoplasmosis or cytomegalovirus infection, in which there is an obliteration of the subarachnoid spaces by fibrous tissue reaction, or to diseases of connective tissue.

IN INFANTS- Head grows at abnormal rate. Anterior fontanel is tense, often bulging, &

non pulsatile. Scalp veins are dilated & markedly so when

infant cries. Macewen’s sign- with increase in

intracranial volume, the bones of the skull become thin & the sutures become palpably separated to produce the cracked pot sound on the percussion of the skull.

Frontal bossing with depressed eyes. Setting-sun sign- eyes rotated downward,

in which sclera may be visible above iris.

Feeds poorly Pupils are sluggish, with unequal response

to light Changes in level of consciousness. Opisthotonus position & lower extremity

spasticity. Cries when picked up & quiets when

allowed to lie still. If hydrocephalus is allowed to progress-

there will be disruption in the lower brainstem function as manifested by difficulty in feeding & a shrill, brief, high-pitched cry. Eventually the skull becomes enlarged, & the cortex is destroyed.

If the condition progress rapidly, the infant may display emesis, somnolence, seizures & cardiopulmonary distress.

IN CHILDHOOD- Headache on awakening with

improvement following emesis or upright posture.

Papilledema, strabismus. Irritable & lethargic. Apathetic, confused & often incoherent. Bulging occiput, nystagmus, ataxia &

cranial nerve palsies.

Routine daily head (occipitofrontal) circumference measurements.

Echoencephalography. A head CT scan is one of the best tests for

identifying hydrocephalus. Arteriography. Brain scan using radioisotopes Cranial ultrasound (an ultrasound of the

brain) Lumbar puncture and examination of the

cerebrospinal fluid (rarely done). Skull x-rays.

The treatment of hydrocephalus is directed toward –

Relief of the hydrocephalus, Treatment of complications, Management of problems related to the

effect of the disorder on psychomotor development. The treatment is, with few exceptions, surgical.

1) MEDICAL MANAGEMENT,2) SURGICAL MANAGEMENT &3) NURSING MANAGEMENT.

This can be tried in mild cases of hydrocephalus.

Acetozolamide: dose of 50mg/kg/day dimnishes CSF production.

Oral glycerol has also been used for the similar purpose.

It may consist of The removal of the obstruction (tumor,

hemorrhage or cyst) to the flow of CSF. Reduction in the amount of CSF produced

through destruction of a portion of the choroid plexus or a third or fourth ventriculostomy.

Shunting of CSF from the ventricle to another site in the normal circulatory passageway of this fluid.

Shunting of CSF from the ventricle to an area outside the CNS, an extracranial body compartment.

Shunting is the most common procedure to be done in the surgical management of hydrocephalus.

Most shunt systems consist of a ventricular catheter, a flush pump, a unidirectional flow valve & a distal catheter. All are radiopaque for easy visualization after placement & all are tested before insertion.

Types of shunts-1.Ventriculoperitonial(VP) shunt.2.Ventriculoatrial(VA) shunt.3.Ventriculopleural shunt.

Ventriculoperitonial(VP) shunt- This is the preferred procedure especially in neonates & young infants. There is greater allowance for excess tubing, which minimizes the number of revisions needed as the child grows. In this ventricular catheter is inserted into the anterior portion of a lateral ventricle through a burr hole in the skull.

An incision is made in abdomen & through the rectus muscle into the peritoneum. The proximal end of the catheter is slipped beneath the skin of anterior abdominal & chest wall to the neck. The ventricular catheter with attached valve is then sutured to the peritoneal catheter. The CSF is absorbed by tissues in the abdominal cavity.

Ventriculoperitonial(VP) shunt-

Ventriculoatrial(VA) shunt- It is reserved for older children who have attained most of their somatic growth & children with abdominal pathology. It requires repeated lengthening as child grows. A silicon catheter is inserted in lateral ventricle & down through the internal jugular vein into left atrium of the heart.

The CSF drains into circulating blood. This type of shunt may become easily obstructed or infected. If an infection occurs, bacterial endocarditis, ventriculitis & bacteremia may result.

Ventriculopleural shunts- these shunts are sometimes used in children over 5 years of age. This type of shunt drains fluid from the lateral ventricle to the pleural cavity. Drainage of the CSF may cause hydrothorax, necessitating either removal of the shunt or a thoracentesis. The nurse must observe these children carefully for respiratory difficulties.

Endoscopic third ventriculostomy- It is a procedure that has potential for greater independence from VP or VA shunting in children with noncommunicating hydrocephalus. In this procedure a small opening is made in the floor of the 3rd ventricle, allowing CSF to flow freely through previously blocked ventricle, thus bypassing the aqueduct of sylvius.

Reports of the success of endoscopic third ventriculostomy in children; however, as surgical techniques & advances continue, it is expected that neonates & small children will be successfully treated with this procedure rather than conventional shunting.

The major complications of shunts are infection & malfunction.

All shunts are subjected to mechanical difficulties, such as kinking, plugging, or separation & migration of tubing.

1) Malfunction is most often caused by mechanical obstruction either within the ventricles from articulate matter (tissue or exudates) or at the distal end from thrombosis or displacement as a result of growth. The child with a shunt obstruction often presents as an emergency with clinical manifestations of increased ICP, frequently accompanied by worsening neurologic status.

2) Shunt infection is also a serious complication of shunts. It can occur at any time, but the period of greatest risk is 1 to 2 months following placement. The infection may be a result of intercurrent infections at the time of shunt replacement. Infections include sepsis, bacterial endocarditis, wound infection, shunt nephritis, meningitis.

3) Another serious shunt-related complication is subdural hematoma caused by rapid reduction of ICP & size.

4) Other complications that may include peritonitis, abdominal abscesses, perforation of abdominal organs by catheter or trochar (at the time of insertion), fistulas, hernias.

NURSING MANAGEMENTA) Teach the family about the management required

for the disorder.a) Treatment is surgical by direct removal of an

obstruction and insertion of shunt to provide primary drainage of the CSF to an extracranial compartment, usually peritoneum (ventriculoperitoneal shunt).

1. The major complications of shunts are infections and malfunction.

2. Other complications include subdural hematoma caused by a too rapid reduction of CSF, peritonitis, abdominal abscess, perforation of organs, fistulas, hernias and ileus.

b. A third ventriculostomy is a new nonshunting procedure used to treat children with hydrocephalus.

NURSING MANAGEMENT contd…B) Provide preoperative nursing carea. Assess head circumference,

fontanelles, cranial sutures, and LOC; check also for irritability, altered feeding habits and a high-pitched cry.

b. Firmly support the head and neck when holding the child.

c. Provide skin care for the head to prevent breakdown.

d. Give small, frequent feedings to decrease the risk of vomiting.

e. Encourage parental-newborn bonding.

NURSING MANAGEMENT contd…C) Provide Postoperative nursing care a. Assess for signs of increased ICP and check the

following; head circumference (daily), anterior fontanels for size and fullness and behaviour.

b. Administer prescribed medications which may include antibiotics to prevent infection and analgesics for pain.

c. If there is increased ICP elevate the head of the bed or allow the child to sit up to enhance gravity flow through shunt.

d. Observe the child for abdominal distension.e. Maintain intake-output chart.

NURSING MANAGEMENT contd…f. Provide shunt care1. Monitor for shunt infection and malfunction which

may be characterized by rapid onset of vomiting, severe headache, irritability, lethargy, fever, redness along the shunt tract, and fluid around the shunt valve.

2. Prevent infection (usually from Staphylococcus epidermis or Staphylococcus aureus)

3. Monitor for shunt overdrainage (headache, dizziness and nausea). Overdrainage may lead to slit ventricle syndrome whereby the ventricle become accustomed to a very small or slitlike configuration, limiting the buffering ability to increased ICP variations

NURSING MANAGEMENT contd…D) Teach home carea. Encourage the child to participate in

age-appropriate activities as tolerated. Encourage the parents to provide as normal lifestyle as possible.

b. Explain how to recognize signs and symptoms of increased ICP. Subtle signs include changes in school performance, intermittent headache, and mild behavior changes

c. Frequent developmental screenings & follow ups.

1) NURSING DIAGNOSIS- Ineffective cerebral tissue perfusion related to increased ICP.

Expected outcome- There will be effective cerebral tissue perfusion.

Interventions: Give proper positioning to the baby. Keep

the head of the baby in neutral (midline) position to promote venous drainage.

Avoid extreme rotation & flexion of the neck as these can cause compression & distortion of jugular veins which will further increase the ICP.

Elevate the head at 30 to 45 degree to aid in venous drainage.

Extreme hip flexion is also avoided as this position causes increase in intra-abdominal & intra-thoracic pressures, which can produce a rise in ICP.

While turning hold the head of baby to avoid any stimuli.

Check for abdominal distension it also results in the rise of ICP.

2) NURSING DIAGNOSIS- Imbalanced nutrition, less than body requirement related to reduced oral intake & vomiting.

Expected outcome- Nutritional status of the baby will be maintained.

Interventions: Assess the nutritional status of the

baby. Weigh the baby daily & record.

Maintain intake-output chart of the baby.

Provide exclusive breast feeding to the neonates & infants up to 6 months of age.

For older children, offer small frequent feedings of light food.

3) NURSING DIAGNOSIS- Risk for impaired skin integrity related to enlarged head.

Expected outcome- Skin integrity will be maintained.

Interventions: Assess the skin integrity of the baby. Prevent the pressure on the enlarged head. Provide firm-soft pillow under child’s head. Keep the area clean & dry. Good skin care & range of motion exercise

are also essential to prevent skin breakdown.

4) NURSING DIAGNOSIS- Risk for infection related to introduction of infecting organism through shunt.

Expected outcome- There will be no sign & symptom of infection.

Interventions: Check & record the vital signs of the baby. Use aseptic technique while doing any

procedure on the baby. Wash the hands properly before touching

the baby. Maintain the general cleanliness of the area

& baby. Administer the antibiotics as prescribed.

5) NURSING DIAGNOSIS- Ineffective family coping related to life threatening problem of infant.

Expected outcome- Family will be able to cope up with the problem.

Interventions: Tell the family about the disease condition

(causes, treatment, prognosis). Educate them about the care of baby like

maintaining the cleanliness, proper diet, skin care, positioning of the baby (initially flat to prevent the excessive CSF drainage then gradual elevation of head of child’s bed to 30 to 45 degree).

Educate the family about the shunt & its purpose.

Tell them about the assessment for excessive drainage of CSF (sunken fontanel, agitation, decrease level of consciousness).

Encourage them for treatment compliance & regular follow up

Marlow R. Dorothy, Redding A. Barbara; Textbook of Pediatric Nursing; sixth edition; Saunders Elsevier; 522-527.

Hockenberry, Wilson; Wong’s Nursing care of infants and children; 7th edition; Mosby; 436-443.

Ghai OP, Paul K Vinod, Bagga Arvind; Ghai essential paediatrics; 7th edition; CBS Publications, 548-549.

Datta Parul; Pediatric Nursing; 2nd edition;Jaypee; 406-409.

 

Singh Meharban; Care of the Newborn; 5th edition; Sagar Publications; 338.

Arvind R; Applied Neonatology; Jaypee; 332-333.

www.ncbi.nlm.nih.gov/hydrocphalus. www.medicinenet.com/hydrocephalus.