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BAGIAN ILMU BEDAH FAKULTAS KEDOKTERAN UNIVERSITAS MULAWARMAN

REFERAT

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 2 2 2 4 4 4 6 6 7 10 11 13 15 17 17 25 26 27 28

Chapter I : Introducing ............................................................................... 1.1. Background 1.2. Aim ........................................................................................

...................................................................................................... ................................................................

Chapter II : Literature Review 2.1. Definition

............................................................................................

2.2. History ................................................................................................. 2.3. Epidemiology ...................................................................................... 2.4. CSF Pathway 2.5. Etiology ....................................................................................

............................................................................................... ................................................................................. .....................................................................

2.6. Pathophysiology

2.7. Clinical Manifestation 2.8. Diagnosis 2.9. Radiology

.......................................................................................... ....................................................................................... ......................................................................

2.10. Differential Diagnosis 2.11. Treatment 2.12. Prevention 2.13. Prognosis

.......................................................................................... ...................................................................................... ........................................................................................... ..................................................................................

Chapter III : Closing References

...................................................................................................

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 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.

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 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-subarachnoidsubgaleal 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 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)

2.5.

Etiology Classification of hydrocephalus (Kaye, 2005) : Obstructive o o o o Lateral ventricle obstruction by tumours (basal ganglia glioma, thalamic glioma) 3rd ventricle obstruction due to colloid cyst Occlusion aqueduct of sylvius 4th ventricular 0bstruction due fossa posterior (medulloblastoma, ependymoma, and acoustic neuroma. Communicating o o o o Obstruction through basal cisterns Failure the absorption of CSF through the arachnoid granulations over the cerebral hemispheres. Infection (bacterial or tubeculosus) Subarachnoid haemorrhage (spontaneous, traumatic or post operative.

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.

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

o o o o

Arnold-Chiari malformation type 1 and type 2 Agenesis of the foramen of Monro Congenital toxoplasmosis 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)

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.

Infections: Meningitis (especially bacterial) and, in some geographic areas, cysticercosis can cause hydrocephalus.

Increased venous sinus pressure: This can be related to achondroplasia, some craniostenoses, or venous thrombosis.

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.

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.

Idiopathic hydrocephalus represents one third of cases of adult hydrocephalus.

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

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.

Prior posterior fossa surgery may cause hydrocephalus by blocking normal pathways of CSF flow.

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 o

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

SAH Head trauma 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 may occur through transventricular absorption of CSF and also by absorption along nerve root sleeves. Temporal and frontal horns dilate first,

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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 Neck pain suggesting tonsillar herniation

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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 98th percentile for age.

o o

Dysjunction of sutures: This can be seen or palpated. Dilated scalp veins: The scalp is thin and shiny with easily visible veins.

Tense fontanelle: The anterior fontanelle in infants who are held erect and are not crying may be excessively tense.

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.

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).

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

o o

Unsteady gait: This is related to spasticity in the lower extremities. Large head: Sutures are closed, but chronic increased ICP will lead to progressive macrocephaly.

Unilateral or bilateral sixth nerve palsy is secondary to increased ICP.

Adults
o o

Papilledema: If raised ICP is not treated, it leads to optic atrophy. Failure of upward gaze and of accommodation indicates pressure on the tectal plate. The full Parinaud syndrome is rare.

Unsteady gait is related to truncal and limb ataxia. Spasticity in legs also causes gait difficulty.

o o

Large head: The head may have been large since childhood. Unilateral or bilateral sixth nerve palsy is secondary to increased ICP.

NPH
o

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

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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.

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.

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.

Ratio of the largest width of the frontal horns to maximal biparietal diameter (ie, Evans ratio) is greater than 30% in hydrocephalus.

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Transependymal exudate is translated on images as periventricular hypoattenuation (CT) or hyperintensity (MRI T2-weighted and fluidattenuated inversion recovery [FLAIR] sequences).

Ballooning of frontal horns of lateral ventricles and third ventricle (ie, "Mickey mouse" ventricles) may indicate aqueductal obstruction.

Upward bowing of the corpus callosum on sagittal MRI suggests acute hydrocephalus.

CT/MRI criteria for chronic hydrocephalus include the following:


o o o o

Temporal horns may be less prominent than in acute hydrocephalus. Third ventricle may herniate into the sella turcica. Sella turcica may be eroded. Macrocrania (ie, occipitofrontal circumference >98th percentile) may be present.

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) 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

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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

Increasing

CSF

reabsorption

Isosorbide

(effectiveness

is

questionable) Surgical Care

Surgical treatment is the preferred therapeutic option.[13]

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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 o

Choroid plexectomy or choroid plexus coagulation may be effective. 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 o

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

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.

A lumboperitoneal shunt is used only for communicating hydrocephalus, CSF fistula, or pseudotumor cerebri.

A Torkildsen shunt is used rarely. It shunts the ventricle to cisternal space and is effective only in acquired obstructive hydrocephalus.

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 o o o

Ventricular tap in infants Open ventricular drainage in children and adults LP in posthemorrhagic and postmeningitic hydrocephalus 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 2.34.6 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 designed neuroendoscopic tools, most straightforward ETV procedures can be performed with a few basic instruments.

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The patient is placed in supine position and the head is elevated to 2030 degrees with slight flexion of the neck. This is done to prevent postoperative pneumocephalus and reduce the risk of subdural 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

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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 10.52.3 mm, whereas the corresponding distance in hydrocephalus patients is 123.7 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. 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.

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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 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

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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.

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)

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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.

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Bibliography

Ahmed, A. S. (2009). Outcome analysis of shunt surgery in hydrocephalus. J Indian Association Pediatric Surgery , 98-101. Alberto, J. H. (2000). www.emedicine.medscape.com. Retrieved September 3, 2012, from www.emedicine.medscape.com: www.emedicine.medscape.com Aschoff, A. K. (1999, October). The scientific history of hydrocephalus and its treatment. Retrieved September 2012, from http://www.ncbi.nlm.nih.gov/pubmed/10547004: http://www.ncbi.nlm.nih.gov/pubmed/10547004 Centers for Disease Control and Prevention. (2011). http://www.cdc.gov/vaccines/vpd-vac/mening/who-vaccinate.htm. . Retrieved 2012, from http://www.cdc.gov/vaccines/vpd-vac/mening/whovaccinate.htm. : http://www.cdc.gov/vaccines/vpd-vac/mening/whovaccinate.htm. Garne, E. L. (2009). Congenital hydrocephalus - prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Europe Journal Pediatric Neurology . Hattigen, E. J.-H. (2010). Diffusion tensor imaging in patients with adult chronic idiopathic hydrocephalus. Neurosurgery , 917-924. Kahlon, B. A. (2007). Is aqueductal stroke volume, measured with cine phasecontrast magnetic resonance imaging scans useful in predicting outcome of shunt surgery in suspected normal pressure hydrocephalus? Neurosurgery , 124-129. Kaye, A. H. (2005). Essential Neurosurgery. Massachusetts: Blackwell Publishing Ltd. Larsson, A. M. (1990). Predictive value of quantitative cisternography in normal pressure hydrocephalus. Acta Neurology Scandinavian , 327-332. Oertel, J. N.-M. (2008). Lower-body parkinsonism: reconsidering the threshold for external lumbar drainage. Nat Clinical Practicional Neurology , 50-55. Persson, E. H. (2005). Hydrocephalus prevalence and outcome in a populationbased cohort of children born in 1989-1998. Acta Pediatric .

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Rekate, H. (2009). A comtemporary definition and classification of hydrocephalus. Semin Pediatric Neurology , 9-15. Satyanegara, H. R. (2010). Ilmu Bedah Saraf. Jakarta, Indonesia: PT. Gramedia Pustaka Utama. Stanley, J. S. (2001, November 15). http//:www.overview-of-hydrocephalus.html. Retrieved September 3, 2012, from http//:www.overview-ofhydrocephalus.html: http//:www.overview-of-hydrocephalus.html Woodworth, G. M. (2009). Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. Neurosurgery , 925-926.

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