hydrocephalus

BAGIAN ILMU BEDAH REFERAT

FAKULTAS KEDOKTERAN

UNIVERSITAS MULAWARMAN

HYDROCEPHALUS

Disusun Oleh :

Sizigia Hascharini Utami

0708015015

Pembimbing :

dr. Arie Ibrahim, Sp. BS

Bagian Ilmu Bedah

Fakultas Kedokteran

Universitas Mulawarman

2012

CONTENTS

Title ................................................................................................................

Contents ....................................................................................................... 1

Chapter I : Introducing ............................................................................... 2

1.1. Background ........................................................................................ 2

1.2. Aim ...................................................................................................... 2

Chapter II : Literature Review ................................................................ 4

2.1. Definition ............................................................................................ 4

2.2. History ................................................................................................. 4

2.3. Epidemiology ...................................................................................... 6

2.4. CSF Pathway .................................................................................... 6

2.5. Etiology ............................................................................................... 7

2.6. Pathophysiology ................................................................................. 10

2.7. Clinical Manifestation ..................................................................... 11

2.8. Diagnosis .......................................................................................... 13

2.9. Radiology ....................................................................................... 15

2.10. Differential Diagnosis ...................................................................... 17

2.11. Treatment .......................................................................................... 17

2.12. Prevention ...................................................................................... 25

2.13. Prognosis ........................................................................................... 26

Chapter III : Closing .................................................................................. 27

References ................................................................................................... 28

1

CHAPTER I

INTRODUCTION

1.1. Background

Hydrocephalus is a condition where in excess of cerebrospinal fluid (CSF)

accumulates within the ventricular system and cisterns of the brain leading

to increased intracranial pressure (ICP) and related consequences.

Hydrocephalus had multifactorial which can affect a fetus, infant, child or

adult. It can describe as imbalance between production and absorption of

CSF. Over production of CSF can caused hydrocephalus due to choroid

plexus tumors, but it is rare. (Ahmed, 2009)

Hydrocephalus is one of the most common clinical conditions affecting the

central nervous system with an incidence of three to for per 1000 births for

congenital hydrocephalus. (Ahmed, 2009)

Congenital hydrocephalus affects about one in every 1000 births. The

overall prevalence in the United States is about 0.5%. Most cases are

detected early, either at or soon after birth. The incidence of acquired

hydrocephalus in adults is not known because it occurs as a result of injury,

illness, or environmental factors. (Stanley, 2001)

In the United states incidence of congenital hydrocephalus is 3 per 1,000

live births; the incidence of acquired hydrocephalus is not known exactly

due to the variety of disorders that may cause it. (Alberto, 2000)

Incidence of acquired hydrocephalus is unknown. About 100,000 shunts are

implanted each year in the developed countries, but little information is

available for other countries. (Alberto, 2000)

On the other literature the prevalence of hydrocephalus was 0.82 per 1000

live births, 0.49 for children with infantile hydrocephalus and 0.33 for

children with myelomeningocoele. The prevalence of infantile

hydrocephalus decreased during the period from 0.55 to 0.43 per 1000. In

2

this group, the aetiology was prenatal in 55% and peri-postnatal in 44% of

the children. The origin was perinatal haemorrhage in all cases born very

preterm. (Persson, 2005)

The incidence of hydrocephalus is a bimodal curve with a peak curve in the

age range of children with various congenital malformations and the rest

associated with normotensive type of adult. Hydrocephalus in adults

reported approximately 40% of all cases. (Satyanegara, 2010)

1.2. Aim

Improve knowledges about definition, pathophysiology, diagnosis,

management, prognosis, and prevention of hydrocephalus.

3

CHAPTER II

LITERATURE REVIEW

2.1. Definition

Hydrocephalus is an abnormal enlargement of the ventricles due to

excessive accumulation of CSF from disturbance of its flow, absorption, or

uncommonly secretion. Normally the volume of CSF is 140 ml. (Kaye,

2005)

Hydrocephalus can be defined broadly as a disturbance of formation, flow,

or absorption of cerebrospinal fluid (CSF) that leads to an increase in

volume occupied by this fluid in the CNS. This condition also could be

termed a hydrodynamic disorder of CSF. (Rekate, 2009)

2.2. History

Hydrocephalus cases were regularly described by Hippocrates, Galen, and

early and medieval Arabian physicians, who believed that this disease was

caused by an extracerebral accumulation of water. Operative procedures

used in ancient times are neither proven by skull findings today nor clearly

reported in the literature. Evacuation of superficial intracranial fluid in

hydrocephalic children was first described in detail in the tenth century by

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Abulkassim Al Zahrawi. In 1744, LeCat published findings on a

ventricular puncture. Effective therapy required aseptic surgery as well as

pathophysiological knowledge--both unavailable before the late nineteenth

century. In 1881, a few years after the landmark study of Key and Retzius,

Wernicke inaugurated sterile ventricular puncture and external CSF

drainage. These were followed in 1891 by serial lumbar punctures

(Quincke) and, in 1893, by the first permanent ventriculo-subarachnoid-

subgaleal shunt (Mikulicz), which was simultaneously a ventriculostomy

and a drainage into an extrathecal low pressure compartment. Between

1898 and 1925, lumboperitoneal, and ventriculoperitoneal, -venous, -

pleural, and -ureteral shunts were invented, but these had a high failure

rate due to insufficient implant materials in most cases. Ventriculostomy

without implants (Anton 1908), with implants, and plexus coagulation

initially had a very high operative mortality and were seldom successful in

the long term, but gradually improved over the next decades. In 1949,

Nulsen and Spitz implanted a shunt successfully into the caval vein with a

ball valve. Between 1955 and 1960, four independent groups invented

distal slit, proximal slit, and diaphragm valves almost simultaneously.

Around 1960, the combined invention of artificial valves and silicone led

to a worldwide therapeutic breakthrough. After the first generation of

simple differential pressure valves, which are unable to drain

physiologically in all body positions, a second generation of adjustable,

autoregulating, antisiphon, and gravitational valves was developed, but

their use is limited due to economical restrictions and still unsolved

technical problems. At the moment, at least 127 different designs are

available, with historical models and prototypes bringing the number to

190 valves, but most of these are only clones. In the 1990s, there has been

a renaissance of endoscopic ventriculostomy, which is widely accepted as

the method of first choice in adult patients with aquired or late-onset,

occlusive hydrocephalus; in other cases the preference remains

controversial. Both new methods, the second generation of valves as well

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as ventriculostomy, show massive deficits in evaluation. There is only one

randomized study and no long-term evaluation. (Aschoff, 1999)

2.3. Epidemiology

Hydrocephalus is one of the most common clinical conditions affecting the

central nervous system with an incidence of three to for per 1000 births for

congenital hydrocephalus. (Ahmed, 2009)

Congenital hydrocephalus affects about one in every 1000 births. The

overall prevalence in the United States is about 0.5%. Most cases are

detected early, either at or soon after birth. The incidence of acquired

hydrocephalus in adults is not known because it occurs as a result of

injury, illness, or environmental factors. (Stanley, 2001)

In the United states incidence of congenital hydrocephalus is 3 per 1,000

live births; the incidence of acquired hydrocephalus is not known exactly

due to the variety of disorders that may cause it. Generally, incidence is

equal in males and females. The exception is Bickers-Adams syndrome, an

X-linked hydrocephalus transmitted by females and manifested in males.

NPH has a slight male preponderance. Hydorcephalus occur on in infancy

and is related to the various forms of congenital malformations and

adulthood, mostly resulting from NPH. (Alberto, 2000)

2.4. CSF Pathway

CSF produced by the choroid plexus in the ventricles 0,4 ml per minute or

about 500 ml in 24 hours. Then CSF flows from lateral ventricles through

foramen of Monro into 3rd ventricle. Then, CSF flows to 4th ventricle

through the aquaduct of Sylvius and through foramina of Magendie and

Luschka into subarachnoid space and basal cisterns. CSF circulates

through subarachnoid space and absorbed by the arachnoid villi of the

dural sinuses. (Kaye, 2005)

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2.5. Etiology

Classification of hydrocephalus (Kaye, 2005) :

Obstructive

o Lateral ventricle obstruction by tumours (basal ganglia glioma,

thalamic glioma)

o 3rd ventricle obstruction due to colloid cyst

o Occlusion aqueduct of sylvius

o 4th ventricular 0bstruction due fossa posterior (medulloblastoma,

ependymoma, and acoustic neuroma.

Communicating

o Obstruction through basal cisterns

o Failure the absorption of CSF through the arachnoid granulations

over the cerebral hemispheres.

o Infection (bacterial or tubeculosus)

o Subarachnoid haemorrhage (spontaneous, traumatic or post

operative.

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o Carcinomatous meningitis that increased CSF viscosity fromhigh

protein content and excessive secretion due to a choroid plexus

papilloma.

Acute hydrocephalus occurs over days, subacute hydrocephalus occurs

over weeks, and chronic hydrocephalus occurs over months or years.

Conditions such as cerebral atrophy and focal destructive lesions also lead

to an abnormal increase of CSF in CNS. In these situations, loss of

cerebral tissue leaves a vacant space that is filled passively with CSF. Such

conditions are not the result of a hydrodynamic disorder and therefore are

not classified as hydrocephalus. An older misnomer used to describe these

conditions was hydrocephalus ex vacuo. (Rekate, 2009)

Normal pressure hydrocephalus (NPH) describes a condition that rarely

occurs in patients younger than 60 years. Enlarged ventricles and normal

CSF pressure at lumbar puncture (LP) in the absence of papilledema led to

the term NPH. However, intermittent intracranial hypertension has been

noted during monitoring of patients in whom NPH is suspected, usually at

night. The classic Hakim triad of symptoms includes gait apraxia,

incontinence, and dementia. (Rekate, 2009; Woodworth, 2009)

Congenital causes in infants and children (Garne, 2009)

o Brainstem malformation causing stenosis of the aqueduct of Sylvius:

This is responsible for 10% of all cases of hydrocephalus in newborns.

o Dandy-Walker malformation: This affects 2-4% of newborns with

hydrocephalus.

o Arnold-Chiari malformation type 1 and type 2

o Agenesis of the foramen of Monro

o Congenital toxoplasmosis

o Bickers-Adams syndrome: This is an X-linked hydrocephalus

accounting for 7% of cases in males. It is characterized by stenosis of

the aqueduct of Sylvius, severe mental retardation, and in 50% by an

adduction-flexion deformity of the thumb.

Acquired causes in infants and children (Alberto, 2000)

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o Mass lesions: Mass lesions account for 20% of all cases of

hydrocephalus in children. These are usually tumors (eg,

medulloblastoma, astrocytoma), but cysts, abscesses, or hematoma also

can be the cause.[6]

o Hemorrhage: Intraventricular hemorrhage can be related to prematurity,

head injury, or rupture of a vascular malformation.

o Infections: Meningitis (especially bacterial) and, in some geographic

areas, cysticercosis can cause hydrocephalus.

o Increased venous sinus pressure: This can be related to achondroplasia,

some craniostenoses, or venous thrombosis.

o Iatrogenic: Hypervitaminosis A, by increasing secretion of CSF or by

increasing permeability of the blood-brain barrier, can lead to

hydrocephalus. As a caveat, hypervitaminosis A is a more common

cause of idiopathic intracranial hypertension, a disorder with increased

CSF pressure but small rather than large ventricles.

o Idiopathic

Causes of hydrocephalus in adults (Oertel, 2008)

o Subarachnoid hemorrhage (SAH) causes one third of these cases by

blocking the arachnoid villi and limiting resorption of CSF. However,

communication between ventricles and subarachnoid space is

preserved.

o Idiopathic hydrocephalus represents one third of cases of adult

hydrocephalus.

o Head injury, through the same mechanism as SAH, can result in

hydrocephalus.

o Tumors can cause blockage anywhere along the CSF pathways. The

most frequent tumors associated with hydrocephalus are ependymoma,

subependymal giant cell astrocytoma, choroid plexus papilloma,

craniopharyngioma, pituitary adenoma, hypothalamic or optic nerve

glioma, hamartoma, and metastatic tumors.

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o Prior posterior fossa surgery may cause hydrocephalus by blocking

normal pathways of CSF flow.

o Congenital aqueductal stenosis causes hydrocephalus but may not be

symptomatic until adulthood. Special care should be taken when

attributing new neurological deficits to congenital hydrocephalus, as its

treatment by shunting may not correct these deficits.

o Meningitis, especially bacterial, may cause hydrocephalus in adults.

o All causes of hydrocephalus described in infants and children are

present in adults who have had congenital or childhood-acquired

hydrocephalus.

Causes of NPH (Most cases are idiopathic and are probably related to a

deficiency of arachnoid granulations.) (Alberto, 2000)

o SAH

o Head trauma

o Meningitis

2.5. Pathophysiology

Normal CSF production is 0.20-0.35 mL/min; most CSF is produced by

the choroid plexus, which is located within the ventricular system, mainly

the lateral and fourth ventricles. The capacity of the lateral and third

ventricles in a healthy person is 20 mL. Total volume of CSF in an adult is

120 mL.

Normal route of CSF from production to clearance is the following: From

the choroid plexus, the CSF flows to the lateral ventricle, then to the

interventricular foramen of Monro, the third ventricle, the cerebral

aqueduct of Sylvius, the fourth ventricle, the 2 lateral foramina of Luschka

and 1 medial foramen of Magendie, the subarachnoid space, the arachnoid

granulations, the dural sinus, and finally into the venous drainage.

ICP rises if production of CSF exceeds absorption. This occurs if CSF is

overproduced, resistance to CSF flow is increased, or venous sinus

pressure is increased. CSF production falls as ICP rises. Compensation

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may occur through transventricular absorption of CSF and also by

absorption along nerve root sleeves. Temporal and frontal horns dilate

first, often asymmetrically. This may result in elevation of the corpus

callosum, stretching or perforation of the septum pellucidum, thinning of

the cerebral mantle, or enlargement of the third ventricle downward into

the pituitary fossa (which may cause pituitary dysfunction).

The mechanism of NPH has not been elucidated completely. Current

theories include increased resistance to flow of CSF within the ventricular

system or subarachnoid villi; intermittently elevated CSF pressure, usually

at night; and ventricular enlargement caused by an initial rise in CSF

pressure; the enlargement is maintained despite normal pressure because

of the Laplace law. Although pressure is normal, the enlarged ventricular

area reflects increased force on the ventricular wall.

2.6. Clinical Manifestation

Clinical features of hydrocephalus are influenced by the following

(Alberto, 2000):

Patient's age

Cause

Location of obstruction

Duration

Rapidity of onset

Symptoms in infants (Alberto, 2000) :

Poor feeding

Irritability

Reduced activity

Vomiting

Symptoms in children (Alberto, 2000) :

Slowing of mental capacity

Headaches (initially in the morning) that are more significant than in

infants because of skull rigidity

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Neck pain suggesting tonsillar herniation

Vomiting, more significant in the morning

Blurred vision: This is a consequence of papilledema and later of optic

atrophy

Double vision: This is related to unilateral or bilateral sixth nerve palsy

Stunted growth and sexual maturation from third ventricle dilatation:

This can lead to obesity and to precocious puberty or delayed onset of

puberty.

Difficulty in walking secondary to spasticity: This affects the lower

limbs preferentially because the periventricular pyramidal tract is

stretched by the hydrocephalus.

Drowsiness

Symptoms in adults (Alberto, 2000) :

Cognitive deterioration: This can be confused with other types of

dementia in the elderly.

Headaches: These are more prominent in the morning because

cerebrospinal fluid (CSF) is resorbed less efficiently in the recumbent

position. This can be relieved by sitting up. As the condition progresses,

headaches become severe and continuous. Headache is rarely if ever

present in normal pressure hydrocephalus (NPH).

Neck pain: If present, neck pain may indicate protrusion of cerebellar

tonsils into the foramen magnum.

Nausea that is not exacerbated by head movements

Vomiting: Sometimes explosive, vomiting is more significant in the

morning.

Blurred vision (and episodes of "graying out"): These may suggest

serious optic nerve compromise, which should be treated as an

emergency.

Double vision (horizontal diplopia) from sixth nerve palsy

Difficulty in walking

Drowsiness

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Incontinence (urinary first, fecal later if condition remains untreated):

This indicates significant destruction of frontal lobes and advanced

disease.

Symptoms of NPH (Alberto, 2000) :

Gait disturbance is usually the first symptom and may precede other

symptoms by months or years. Magnetic gait is used to emphasize the

tendency of the feet to remain "stuck to the floor" despite patients’ best

efforts to move them.

Dementia should be a late finding in pure (shunt-responsive) NPH. It

presents as an impairment of recent memory or as a "slowing of

thinking." Spontaneity and initiative are decreased. The degree can vary

from patient to patient.

Urinary incontinence may present as urgency, frequency, or a

diminished awareness of the need to urinate.

Other symptoms that can occur include personality changes and

Parkinsonism. Seizures are extremely rare and should prompt

consideration for an alternative diagnosis.

2.7. Diagnosis

Physical examination (Alberto, 2000)

Infants

o Head enlargement: Head circumference is at or above the 98 th

percentile for age.

o Dysjunction of sutures: This can be seen or palpated.

o Dilated scalp veins: The scalp is thin and shiny with easily visible

veins.

o Tense fontanelle: The anterior fontanelle in infants who are held

erect and are not crying may be excessively tense.

o Setting-sun sign: In infants, it is characteristic of increased

intracranial pressure (ICP). Ocular globes are deviated downward,

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the upper lids are retracted, and the white sclerae may be visible

above the iris.

o Increased limb tone: Spasticity preferentially affects the lower limbs.

The cause is stretching of the periventricular pyramidal tract fibers

by hydrocephalus.

Children

o Papilledema: if the raised ICP is not treated, this can lead to optic

atrophy and vision loss.

o Failure of upward gaze: This is due to pressure on the tectal plate

through the suprapineal recess. The limitation of upward gaze is of

supranuclear origin. When the pressure is severe, other elements of

the dorsal midbrain syndrome (ie, Parinaud syndrome) may be

observed, such as light-near dissociation, convergence-retraction

nystagmus, and eyelid retraction (Collier sign).

o Macewen sign: A "cracked pot" sound is noted on percussion of the

head.

o Unsteady gait: This is related to spasticity in the lower extremities.

o Large head: Sutures are closed, but chronic increased ICP will lead

to progressive macrocephaly.

o Unilateral or bilateral sixth nerve palsy is secondary to increased

ICP.

Adults

o Papilledema: If raised ICP is not treated, it leads to optic atrophy.

o Failure of upward gaze and of accommodation indicates pressure on

the tectal plate. The full Parinaud syndrome is rare.

o Unsteady gait is related to truncal and limb ataxia. Spasticity in legs

also causes gait difficulty.

o Large head: The head may have been large since childhood.

o Unilateral or bilateral sixth nerve palsy is secondary to increased

ICP.

NPH

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o Muscle strength is usually normal. No sensory loss is noted.

o Reflexes may be increased, and the Babinski response may be found

in one or both feet. These findings should prompt search for vascular

risk factors (causing associated brain microangiopathy or vascular

Parkinsonism), which are common in NPH patients.

o Difficulty in walking varies from mild imbalance to inability to walk

or to stand. The classic gait impairment consists of short steps, wide

base, externally rotated feet, and lack of festination (hastening of

cadence with progressively shortening stride length, a hallmark of

the gait impairment of Parkinson disease). These abnormalities may

progress to the point of apraxia. Patients may not know how to take

steps despite preservation of other learned motor tasks.

o Frontal release signs such as sucking and grasping reflexes appear in

late stages.

2.8. Radiology

The most important investigation is either a CT scan or MRI of the brain

which will show which ventricles are dilated. If the lateral ventricles and

3rd ventricles are all very dilated and 4th ventricle is small, the obstruction

at the level aqueduct of Sylvius. (Kaye, 2005)

Magnetic resonance imaging. In the sagital plane MRI is particularly

helpful in showing aqueduct. USG (Ultrasonography) through open

anterior fontanelle is useful in assesing ventricular size in infants and may

obviate the need for repeated CT scans. Plain skull X-ray can demonstrate

splayed suture, erosion of the bony buttresses around the tuberculum sellae

or a ‘copper beaten’ appeareance to the inside of the calvarium. (Kaye,

2005)

CT/MRI criteria for acute hydrocephalus include the following:

o Size of both temporal horns is greater than 2 mm, clearly visible. In the

absence of hydrocephalus, the temporal horns should be barely visible.

15

o Ratio of the largest width of the frontal horns to maximal biparietal

diameter (ie, Evans ratio) is greater than 30% in hydrocephalus.

o Transependymal exudate is translated on images as periventricular

hypoattenuation (CT) or hyperintensity (MRI T2-weighted and fluid-

attenuated inversion recovery [FLAIR] sequences).

o Ballooning of frontal horns of lateral ventricles and third ventricle (ie,

"Mickey mouse" ventricles) may indicate aqueductal obstruction.

o Upward bowing of the corpus callosum on sagittal MRI suggests acute

hydrocephalus.

CT/MRI criteria for chronic hydrocephalus include the following:

o Temporal horns may be less prominent than in acute hydrocephalus.

o Third ventricle may herniate into the sella turcica.

o Sella turcica may be eroded.

o Macrocrania (ie, occipitofrontal circumference >98th percentile) may be

present.

o Corpus callosum may be atrophied (best appreciated on sagittal MRI).

In this case, parenchymal atrophy and ex-vacuo (rather than true)

hydrocephalus from a neurodegenerative disease should be considered.

Ultrasonography through the anterior fontanelle in infants is useful for

evaluating subependymal and intraventricular hemorrhage and in

following infants for possible development of progressive hydrocephalus.

Radionuclide cisternography can be done in NPH to evaluate the prognosis

with regard to possible shunting. If a late scan (48-72 h) shows persistence

of ventricular activity with a ventricular to total intracranial activity (V/T

ratio) greater than 32%, the patient is more likely to benefit from shunting.

Because of its poor sensitivity in predicting shunt response when the V/T

ration is less than 32%, this test is no longer commonly used.

Skull radiographs may depict erosion of sella turcica, or "beaten copper

cranium" (called by some authors "beaten silver cranium"). The latter can

also be seen in craniosynostosis. (Larsson, 1990)

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MRI cine is an MRI technique to measure CSF stroke volume (SV) in the

cerebral aqueduct. Cine phase-contrast MRI measurements of SV in the

cerebral aqueduct does not appear to be useful in predicting response to

shunting. (Kahlon, 2007)

Diffusion tensor imaging (DTI) is a novel imaging technique that detects

differences in fractional anisotropy (FA) and mean diffusivity (MD) of the

brain parenchyma surrounding the ventricles. Impairment of FA and MD

through DTI allows the recognition of microstructural changes in

periventricular white matter region that may be too subtle on conventional

MRI. (Hattigen, 2010)

2.9. Differential Diagnosis

Intracranial Hemorrhage

Pediatric idiopatic intracranial hypertension

Pseudotumor cerebri

Subdural Empyema

2.10. Treatment

Medical Care

Medical treatment in hydrocephalus is used to delay surgical

intervention. It may be tried in premature infants with posthemorrhagic

hydrocephalus (in the absence of acute hydrocephalus). Normal CSF

absorption may resume spontaneously during this interim period.

Medical treatment is not effective in long-term treatment of chronic

hydrocephalus. It may induce metabolic consequences and thus should

be used only as a temporizing measure.

Medications affect CSF dynamics by the following mechanisms:

o Decreasing CSF secretion by the choroid plexus - Acetazolamide

and furosemide

o Increasing CSF reabsorption - Isosorbide (effectiveness is

questionable)

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Surgical Care

Surgical treatment is the preferred therapeutic option.[13]

Repeat lumbar punctures (LPs) can be performed for cases of

hydrocephalus after intraventricular hemorrhage, since this condition

can resolve spontaneously. If reabsorption does not resume when the

protein content of cerebrospinal fluid (CSF) is less than 100 mg/dL,

spontaneous resorption is unlikely to occur. LPs can be performed only

in cases of communicating hydrocephalus.

Alternatives to shunting include the following:

o Choroid plexectomy or choroid plexus coagulation may be effective.

o Opening of a stenosed aqueduct has a higher morbidity rate and a

lower success rate than shunting, except in the case of tumors.

However, lately cerebral aqueductoplasty has gained popularity as an

effective treatment for membranous and short-segment stenoses of

the sylvian aqueduct. It can be performed through a coronal

approach or endoscopically through suboccipital foramen magnum

trans-fourth ventricle approach.

o In these cases, tumor removal cures the hydrocephalus in 80%.

o Endoscopic fenestration of the floor of the third ventricle establishes

an alternative route for CSF toward the subarachnoid space. It is

contraindicated in communicating hydrocephalus.

Shunts eventually are performed in most patients. Only about 25% of

patients with hydrocephalus are treated successfully without shunt

placement. The principle of shunting is to establish a communication

between the CSF (ventricular or lumbar) and a drainage cavity

(peritoneum, right atrium, pleura). Remember that shunts are not

perfect and that all alternatives to shunting should be considered first.

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o A ventriculoperitoneal (VP) shunt is used most commonly. The

lateral ventricle is the usual proximal location. The advantage of this

shunt is that the need to lengthen the catheter with growth may be

obviated by using a long peritoneal catheter.

o A ventriculoatrial (VA) shunt also is called a "vascular shunt." It

shunts the cerebral ventricles through the jugular vein and superior

vena cava into the right cardiac atrium. It is used when the patient

has abdominal abnormalities (eg, peritonitis, morbid obesity, or after

extensive abdominal surgery). This shunt requires repeated

lengthening in a growing child.

o A lumboperitoneal shunt is used only for communicating

hydrocephalus, CSF fistula, or pseudotumor cerebri.

o A Torkildsen shunt is used rarely. It shunts the ventricle to cisternal

space and is effective only in acquired obstructive hydrocephalus.

o A ventriculopleural shunt is considered second line. It is used if

other shunt types are contraindicated.

Rapid-onset hydrocephalus with increased intracranial pressure (ICP) is

an emergency. The following can be done, depending on each specific

case:

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o Ventricular tap in infants

o Open ventricular drainage in children and adults

o LP in posthemorrhagic and postmeningitic hydrocephalus

o VP or VA shunt

Neuroendoscopy

Endoscopic surgical systems have undergone revolutionary changes in

the last two decades, thanks to technological advancements such as rod

lens systems, fiber optic technology, and better illumination with

powerful light sources and high resolution.

Neuroendoscopic systems can be divided into two main categories:

rigid and flexible endoscopes. These have different indications for use,

and each has its own advantages and disadvantages. In rigid

endoscopes,

the view angles vary from 0 to120 degrees. Those with 0-30 degree

view angles provide appropriate optical and anatomical orientation for

straightforward cases.

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The outer diameters of rigid endoscopes are usually 3.8-6.2 mm, but

may be larger or smaller depending on the endoscope used. The main

advantages of rigid endoscopes over flexible endoscopes are better

image quality, wider and multiple working channels, stability, and

adaptability to stereotactic frames. The disadvantages of these

instruments are larger diameter and limited maneuverability. Flexible

endoscopes are thinner and less traumatic than rigid endoscopes. Their

outer diameter is 2.3·4.6 mm, and their main advantage is superior

maneuverability. The main disadvantages of these scopes are narrower

working channels and poor image quality.

The neuroendoscopic armamentarium has expanded continuously

during the last decade. The most widely used and specially designed

neuroendoscopic instruments are probe-perforators, Fogarty catheters,

biopsy and grasping forceps, scissors, mono- and bipolar cauteries,

suction tips, and laser wires (4,6). Although there are many specially

21

designed neuroendoscopic tools, most straightforward ETV procedures

can be performed with a few basic instruments.

The patient is placed in supine position and the head is elevated to 20-

30 degrees with slight flexion of the neck. This is done to prevent

postoperative pneumocephalus and reduce the risk of subdural

22

hematoma. An incision is made in the scalp and a burr-hole is drilled on

or just in front of the coronal suture on the mid-pupillary line. The

optimal entry point for ETVwas found as 8mm anterior to the coronal

suture and 28 mm lateral to the midline in a study. After a burr-hole of

approximately 1cm diameter is created, the dura is opened in cruciate

fashion and a peel-away cannula (12F) or rigid sheath (7 111m),

depending on the endoscopic system used, is introduced into the frontal

horn of the lateral ventricle. The endoscope is then passed through the

cannula into the frontal horn. The foramen of Monro is located by

following the choroid plexus, anterior septal, and thalamostriate veins,

and the endoscope is passed through this opening and placed into the

third ventricle. In normal subjects, the mean sagittal diameter of the

foramen of Monro is 2.9 mm and the vertical diameter is 5.1 mm. This

foramen is usually considerably enlarged in hydrocephalic patients, and

the endoscope can usually pass through easily without injuring the

fornix. Once the endoscope is in the third ventricle, the infundibular

recess, tuber cinereum, mamillary bodies, massa intermedia, aqueduct,

and posterior commissure can be observed from anterior to posterior.

The optic recess, lamina terminalis, and suprapineal recess can be seen

if the instrument is a wide angled rigid or flexible endoscope.

Fenestration is performed at the tuber cinereum at the midway between

the infundibular recess and the intermamillary point. Ideally, the site of

fenestration should be away from the basillary tip. onnally, the mean

distance between the infundibular recess and mamillary bodies is 6mm

(range,3.5-9mm). The mean distance between the basillary artery and

the infundibular recess in the normal setting is 10.5±2.3 mm, whereas

the corresponding distance in hydrocephalus patients is 12±3.7 mm. If

the ventricle floor is translucent, the basilar artery may be seen and

fenestration is performed distant from it. It is also critical to fenestrate

at the above-mentioned mid-point, because more lateral fenestration

may cause a third nerve injury.

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Fenestration of the floor of the third ventricle may be performed using a

blunt probe, Fogarty catheter, the endoscope itself, special scissors, a

coagubtor, or a number of other instruments, depending on the

surgeon's preference. We use an angled blunt probe designed for this

purpose, and angle the tip of the probe toward the dorsum sella so as

not to injure the basilar artery during fenestration. As mentioned above,

the floor of the ventricle is usually quite thin in patients with

hydrocephalus, and can be easily punctured with a blunt probe.

However, in some cases it may be relatively thick, and the surgeon may

prefer to use coagulation or sharp fenestration techniques in these cases.

However, I do not recommend using coagulation to fenestrate the floor,

as this may damage vascular structures below and may cause thermal

injury to the hypothalamus.

After the floor of the third ventricle is punctured, . the fenestrated site is

enlarged using a 3F Fogarty catheter. The catheter is passed through the

puncture hole, its balloon is inflated, and the catheter is then withdrawn

to enlarge the hole. Using this method, a fenestration of 5-6 mm

diameter is created. It is important to remember that the Fogarty

catheter may injure vascular structures and the third cranial nerve

below, and should not be advanced into the prepontine space too much.

The proximal end of the balloon should be visible to the surgeon. Once

this enlarged passageway is formed, the endoscope is inserted into the

prepontine space to explore the basilar artery and its tributaries, the

pons, the dorsum, and the clivus. It is not uncommon to observe a

second membrane, often connected to the Lilliquest membrane, in the

prepontine space. The main purpose of this exploration is to ensure

there is no other membrane obstructing free CSF flow in the prepontine

space. If there is such an obstructing membrane, it must also be

fenestrated with a blunt probe and enlarged with a Fogarty catheter, as

described above. After the prepontine space has been explored, the

endoscope is withdrawn into the third ventricle and the examiner will

24

observe pulsations of the floor along with "flapping" of the edges of the

newly created opening as CSF flows through indicating a patent

ventriculostomy.

It is not unusual to observe some bleeding during fenestration,

especially if the floor is thick and vascular. However, this is easily

stopped by irrigating the field with Ringer's lactate for awhile. Another

way to stop hemorrhage from the edges of the new opening is to inflate

the Fogarty balloon just at the level of the opening so that it compresses

the edges. The inflated balloon should be kept in place for 15-30

seconds. When the procedure is complete, the endoscope is withdrawn

slowly, exploring the third and lateral ventricles to ensure there is no

acti ve bleeding. A piece of Gelfoam® is placed in the burr-hole and the

scalp is closed in standard fashion. Some surgeons leave a ventricular

drain in place after ETV. The purpose of this is to measure intracranial

pressure (ICP) and be able to drain CSF if necessary. At our center, we

do not place a ventricular drain if there are no peroperative problems.

2.11. Prevention

There are no known ways to prevent all cases of hydrocephalus. In general

:

Get regular prenatal care.

Protect yourself or your child from head injuries.

Keep your child's vaccines up to date.

Preliminary research suggests that some cases due to brain bleeding in the

newborn period may be preventable. Cytomegalovirus or toxoplasmosis

acquired by a mother during pregnancy may be a cause of hydrocephalus

in a newborn baby. Mothers may reduce their risk of being infected with

toxoplasmosis with these steps:

Carefully cook meat and vegetables.

Correctly clean contaminated knives and cutting surfaces.

Avoid handling cat litter, or wear gloves when cleaning the litter box.

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Pet rodents (mice, rats, hamsters) often carry a virus called lymphocytic

choriomeningitis virus (LCV). LCV infection acquired from pets during

pregnancy can lead to hydrocephalus. This is preventable by avoiding

rodent contact. (Centers for Disease Control and Prevention, 2011)

Infection with Chickenpox or Mumps during or immediately after

pregnancy may also lead to hydrocephalus in the baby. Both of these

infections can be prevented with vaccination. Other preventable infections

may also cause hydrocephalus. People who have risk factors for

hydrocephalus should be carefully monitored. Immediate treatment might

prevent long-term complications. (Centers for Disease Control and

Prevention, 2011)

2.12. Prognosis

Long-term outcome is related directly to the cause of hydrocephalus. Up to

50% of patients with large intraventricular hemorrhage develop permanent

hydrocephalus requiring shunt. Following removal of a posterior fossa

tumor in children, 20% develop permanent hydrocephalus requiring a

shunt. The overall prognosis is related to type, location, and extent of

surgical resection of the tumor. Satisfactory control was reported for

medical treatment in 50% of hydrocephalic patients younger than 1 year

who had stable vital signs, normal renal function, and no symptoms of

elevated ICP.

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CHAPTER III

CLOSING

3.1. Conclusions

Hydrocephalus is a condition where in excess of cerebrospinal fluid

(CSF) accumulates within the ventricular system and cisterns of the

brain leading to increased intracranial pressure (ICP) and related

consequences. Hydrocephalus had multifactorial which can affect a

fetus, infant, child or adult. It can describe as imbalance between

production and absorption of CSF.

The aimed of treatment hyrocephalus was to evacuated the LCS or

remove the obstruction.

Long-term outcome is related directly to the cause of hydrocephalus.

27

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