Hydrocephalus project

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HydrocephalusHydrocephalusin Paediatricin Paediatric

By: By:

Hieder A`alaHieder A`ala

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Cerebro-Spinal Fluid (CSF)Cerebro-Spinal Fluid (CSF)

Definition: Definition: is a clear bodily fluid that occupies the subarachnoid space in the brain (the space between the skull and the cerebral cortex—more specifically, between the arachnoid and pia layers of the meninges). It is a very pure saline solution with microglia and acts as a "cushion" or buffer

for the cortex.

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PhysiologyPhysiology Cerebrospinal fluid also occupies the Cerebrospinal fluid also occupies the ventricular systemventricular system of the of the

brain and the brain and the spinal cordspinal cord. It is a prime example of the . It is a prime example of the separation of brain function from the rest of the body, as all separation of brain function from the rest of the body, as all CSF is generated locally in the brain. It is produced by the CSF is generated locally in the brain. It is produced by the choroid plexuschoroid plexus which is formed by specialized ependymal which is formed by specialized ependymal cells. The cells. The choroid plexuschoroid plexus enter the lateral ventricles through enter the lateral ventricles through the choroid fissure, along the line of the fimbria/fornix, and the choroid fissure, along the line of the fimbria/fornix, and the third and fourth ventricle through their roofs. The CSF the third and fourth ventricle through their roofs. The CSF formed by the choroid plexuses in the ventricles, circulates formed by the choroid plexuses in the ventricles, circulates through the interventricular foramina (foramen of Monro) into through the interventricular foramina (foramen of Monro) into the third ventricle and then via the mesencephalic duct the third ventricle and then via the mesencephalic duct (cerebral aqueduct) into the fourth ventricle, whence it exits (cerebral aqueduct) into the fourth ventricle, whence it exits through two lateral apertures (foramina of Luschka) and one through two lateral apertures (foramina of Luschka) and one median aperture (foramen of Magendie). It then flows through median aperture (foramen of Magendie). It then flows through the cerebromedullary cistern down the spinal cord and over the cerebromedullary cistern down the spinal cord and over the cerebral hemispheres.the cerebral hemispheres.

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PhysiologyPhysiology Traditionally, it has been thought that CSF returns to the vascular system Traditionally, it has been thought that CSF returns to the vascular system

by entering the dural venous sinuses via the arachnoid granulations. by entering the dural venous sinuses via the arachnoid granulations. However, some have suggested that CSF flow along the cranial nerves and However, some have suggested that CSF flow along the cranial nerves and spinal nerve roots allow it into the lymphatic channels and that this flow spinal nerve roots allow it into the lymphatic channels and that this flow may play a substantial role in CSF reabsorbtion, particularly in the neonate may play a substantial role in CSF reabsorbtion, particularly in the neonate (in which arachnoid granulations are sparsely distributed). (in which arachnoid granulations are sparsely distributed).

The cerebrospinal fluid is produced by the ventricles (mostly the lateral The cerebrospinal fluid is produced by the ventricles (mostly the lateral ventricles) at a rate of 500 ml/day. Since the volume that may be contained ventricles) at a rate of 500 ml/day. Since the volume that may be contained by the brain is of 150 ml, it is frequently replaced (3-4 times per day by the brain is of 150 ml, it is frequently replaced (3-4 times per day turnover), exceeding amounts getting into the blood. This continuous flow turnover), exceeding amounts getting into the blood. This continuous flow through the ventricular system into the subarachnoid space and finally through the ventricular system into the subarachnoid space and finally exiting into the venous system provides somewhat of a "sink" that reduces exiting into the venous system provides somewhat of a "sink" that reduces the concentration of larger, lipoinsoluble molecules penetrating into the the concentration of larger, lipoinsoluble molecules penetrating into the brain and CSF. brain and CSF.

The CSF contains approximately 0.3% plasma proteins, also being 15 to 40 The CSF contains approximately 0.3% plasma proteins, also being 15 to 40 mg/dL, depending on sampling site. mg/dL, depending on sampling site.

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What is Hydrocephalus?What is Hydrocephalus?

The term hydrocephalus is derived from the Greek words The term hydrocephalus is derived from the Greek words “hydro” meaning water and “cephalus” meaning head. As the “hydro” meaning water and “cephalus” meaning head. As the name implies, (water head) it is a condition in which the name implies, (water head) it is a condition in which the primary characteristic is excessive accumulation of fluid in the primary characteristic is excessive accumulation of fluid in the brain. The excessive accumulation of (CSF) cerebrospinal brain. The excessive accumulation of (CSF) cerebrospinal fluid results in an abnormal dilatation of the spaces in the fluid results in an abnormal dilatation of the spaces in the brain called ventricles. This dilatation causes potentially brain called ventricles. This dilatation causes potentially harmful pressure on the tissue of the brain.harmful pressure on the tissue of the brain.

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Aetiology of hydrocephalusAetiology of hydrocephalus

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classifications of Hydrocephalusclassifications of Hydrocephalus

We will classify causes of hydrocephalus into We will classify causes of hydrocephalus into two forms according to two principles.two forms according to two principles.

1-according to relation or comminucation 1-according to relation or comminucation between the ventricular system and the venous between the ventricular system and the venous sinuses (i.e. obstructive or non-obstructive).sinuses (i.e. obstructive or non-obstructive).

2-according to the nature of the aetiology 2-according to the nature of the aetiology whether acquired or congenital.whether acquired or congenital.

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11stst classification classificationA-Obstructive (non-communicating)A-Obstructive (non-communicating)

This type of hydrocephalus This type of hydrocephalus results from an obstruction results from an obstruction within the ventricular within the ventricular system of the brain that system of the brain that prevents CSF from flowing prevents CSF from flowing or “communicating” within or “communicating” within the brain. the brain. The most The most common type is a narrowing common type is a narrowing of a channel in the brain that of a channel in the brain that connects two ventricles connects two ventricles together. together.

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11stst classification classificationB-Non-obstructive (communicating)B-Non-obstructive (communicating)

This type results from This type results from problems with the problems with the production or production or absorption of CSF. absorption of CSF. The The most common is caused most common is caused by bleeding into the by bleeding into the subarachnoid space in subarachnoid space in the brain.the brain.

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22ndnd classification classificationA-A-Congenital causes in infants and childrenCongenital causes in infants and children

Stenoses of the aqueduct of Sylvius due to malformation: This is Stenoses of the aqueduct of Sylvius due to malformation: This is responsible for 10% of all cases of hydrocephalus in newborns.responsible for 10% of all cases of hydrocephalus in newborns.

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

Arnold-Chiari malformation type 1 and type 2 and type threeArnold-Chiari malformation type 1 and type 2 and type three Agenesis of the foramen of MonroAgenesis of the foramen of Monro Congenital toxoplasmosisCongenital toxoplasmosis Bickers-Adams syndrome: This is an X-linked hydrocephalus Bickers-Adams syndrome: This is an X-linked hydrocephalus

accounting for 7% of cases in males. It is characterized by accounting for 7% of cases in males. It is characterized by stenosis of the aqueduct of Sylvius, severe mental retardation, stenosis of the aqueduct of Sylvius, severe mental retardation, and in 50% by an adduction-flexion deformity of the thumband in 50% by an adduction-flexion deformity of the thumb

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22ndnd classification classification B-Acquired causes in infants and childrenB-Acquired causes in infants and children

Mass lesions account for 20% of all cases of hydrocephalus in Mass lesions account for 20% of all cases of hydrocephalus in children. These are usually tumors (eg, medulloblastoma, children. These are usually tumors (eg, medulloblastoma, astrocytoma), but cysts, abscesses, or hematoma also can be the cause.astrocytoma), but cysts, abscesses, or hematoma also can be the cause.

Intraventricular hemorrhage can be related to prematurity, head injury, Intraventricular hemorrhage can be related to prematurity, head injury, or rupture of a vascular malformation.or rupture of a vascular malformation.

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

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

Iatrogenic: Hypervitaminosis A, by increasing secretion of CSF or by Iatrogenic: Hypervitaminosis A, by increasing secretion of CSF or by increasing permeability of the blood-brain barrier, can lead to increasing permeability of the blood-brain barrier, can lead to hydrocephalus.hydrocephalus.

IdiopathicIdiopathic

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Some important definitionsSome important definitions

CHIARI MALFORMATIONCHIARI MALFORMATION TYPE I: TYPE I:

DISPLACEMENT OF CEREBELLAR TONSILS INTO THE DISPLACEMENT OF CEREBELLAR TONSILS INTO THE CERVICAL CANAL.CERVICAL CANAL.

GIVES SYMPTOMS IN ADOLESCENCE OR ADULT LIFE. GIVES SYMPTOMS IN ADOLESCENCE OR ADULT LIFE. (HEADACHE, NECK PAIN)(HEADACHE, NECK PAIN)

NO HYDROCEPHALUSNO HYDROCEPHALUS

TYPE II :TYPE II : DISPLACEMENT OF INFERIOR VERMIS, PONS, AND DISPLACEMENT OF INFERIOR VERMIS, PONS, AND

MEDULLA INTO CERVICAL CANAL MEDULLA INTO CERVICAL CANAL PROGRESSIVE HYDROCEPHALUS AND PROGRESSIVE HYDROCEPHALUS AND

MYELOMENINGOCELE. MYELOMENINGOCELE. ELONGATION OF THE 4TH VENTRICLE.ELONGATION OF THE 4TH VENTRICLE.

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DANDY-WALKER SYNDROMEDANDY-WALKER SYNDROME1.1. CYSTIC EXPANSION OF THE 4TH CYSTIC EXPANSION OF THE 4TH

VENTRICLE IN THE POSTERIOR VENTRICLE IN THE POSTERIOR FOSSA.FOSSA.

2.2. DEVELOPMENTAL FAILURE OF ROOF DEVELOPMENTAL FAILURE OF ROOF OF 4TH VENTRICLE DURING OF 4TH VENTRICLE DURING EMBRYOGENESIS.EMBRYOGENESIS.

3.3. 90 % HAVE HYDROCEPHALUS90 % HAVE HYDROCEPHALUS4.4. PROMINENT OCCIPUTPROMINENT OCCIPUT

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Other types of HydrocephalusOther types of Hydrocephalus

Two other forms of hydrocephalus which don’t fit distinctly Two other forms of hydrocephalus which don’t fit distinctly into those categories are into those categories are hydrocephalus ex-vacuohydrocephalus ex-vacuo, and , and normal normal pressure hydrocephaluspressure hydrocephalus. .

N.B.:N.B.: ** Ex-vacuo occurs when there is damage to the brain Ex-vacuo occurs when there is damage to the brain caused by stroke of a traumatic injury.caused by stroke of a traumatic injury.

** Normal pressure hydrocephalus commonly occurs in Normal pressure hydrocephalus commonly occurs in the elderly, due to aging. The triad (Hakim triad) of gait the elderly, due to aging. The triad (Hakim triad) of gait instability, urinary incontinence and dementia is a relatively instability, urinary incontinence and dementia is a relatively typical manifestation of the distinct entity normal pressure typical manifestation of the distinct entity normal pressure hydrocephalus (NPH). The triad can easily be remembered as hydrocephalus (NPH). The triad can easily be remembered as "Wacky, Wet, and Wobbly!" "Wacky, Wet, and Wobbly!"

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IVHIVH DEFINITION:DEFINITION:

BLEEDING IN SUBEPENDIMAL GERMINAL BLEEDING IN SUBEPENDIMAL GERMINAL MATRIX WITH/WITHOUT EXTENSION INTO MATRIX WITH/WITHOUT EXTENSION INTO VENTRICLES AND BRAIN PARENCHYMAVENTRICLES AND BRAIN PARENCHYMA

INCIDENCE:INCIDENCE: IN PREMATURES 25 - 40 %IN PREMATURES 25 - 40 %

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PATHOLOGY:PATHOLOGY: INTRAVASCULARINTRAVASCULAR VASCULARVASCULAR EXTRAVASCULAREXTRAVASCULAR

COMPLICATIONS:COMPLICATIONS: HYDROCEPHALUS HYDROCEPHALUS

20 % IN MODERATE BLEEDS20 % IN MODERATE BLEEDS 65-100 % IN LARGE BLEEDS65-100 % IN LARGE BLEEDS

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PathophysiologyPathophysiology

Pathological basis of clinical picture:Pathological basis of clinical picture:1.1. increased intracranial tension (ICP).increased intracranial tension (ICP).2.2. Age of onset.Age of onset.3.3. Type of hydrocephalus (obs. or non obs.)Type of hydrocephalus (obs. or non obs.)4.4. Closure of fontanels and sutures of the skullClosure of fontanels and sutures of the skull5.5. Associated disorders.Associated disorders.6.6. Treatment.( relapsing of the symptoms due Treatment.( relapsing of the symptoms due

to complication associated with treatment )to complication associated with treatment )

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PathophysiologyPathophysiology ICP rises if production of CSF exceeds absorption. ICP rises if production of CSF exceeds absorption.

This occurs if CSF is overproduced, resistance to This occurs if CSF is overproduced, resistance to CSF flow is increased, or venous sinus pressure is CSF flow is increased, or venous sinus pressure is increased. CSF production falls as ICP rises. increased. CSF production falls as ICP rises. Compensation may occur through transventricular Compensation may occur through transventricular absorption of CSF and also by absorption along nerve absorption of CSF and also by absorption along nerve root sleeves. Temporal and frontal horns dilate first, root sleeves. Temporal and frontal horns dilate first, often asymmetrically. This may result in elevation of often asymmetrically. This may result in elevation of the corpus callosum, stretching or perforation of the the corpus callosum, stretching or perforation of the septum pellucidum, thinning of the cerebral mantle, septum pellucidum, thinning of the cerebral mantle, or enlargement of the third ventricle downward into or enlargement of the third ventricle downward into the pituitary fossa (which may cause pituitary the pituitary fossa (which may cause pituitary dysfunction). dysfunction).

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Age of onset Age of onset There are three groups in which hydrocephalus There are three groups in which hydrocephalus

can develop:can develop:1.1. Fetuses (diagnosed antenataly ) Fetuses (diagnosed antenataly ) 2.2. Infants .Infants .3.3. Children.Children.Each group has its own criteria of hydrocephalusEach group has its own criteria of hydrocephalus Type of hydrocephalus (obs.or non obs.)Type of hydrocephalus (obs.or non obs.)

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Closure of fontanels and sutures of the skull :Closure of fontanels and sutures of the skull :

As there is limited space for expansion in the As there is limited space for expansion in the skull, CSF pressure (as total intracranial skull, CSF pressure (as total intracranial pressure) effects the arterial profusion to the pressure) effects the arterial profusion to the brain. When CSF pressure is elevated, cerebral brain. When CSF pressure is elevated, cerebral blood flow may be diminished .blood flow may be diminished .

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Associated disorders. ( see later )Associated disorders. ( see later )

Meningitis, ventriculaitis , meningeocele , Meningitis, ventriculaitis , meningeocele , cerebellar herniation ..etc.cerebellar herniation ..etc.

Treatment as option, time of intervention , Treatment as option, time of intervention , modality , complications and rate of modality , complications and rate of recurrence .recurrence .

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Pathophysiology Pathophysiology

As there is limited space for expansion in the As there is limited space for expansion in the skull, CSF pressure (as total intracranial skull, CSF pressure (as total intracranial pressure) effects the arterial profusion to the pressure) effects the arterial profusion to the brain. When CSF pressure is elevated, cerebral brain. When CSF pressure is elevated, cerebral blood flow may be diminished .blood flow may be diminished .

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Mortality/MorbidityMortality/Morbidity Mortality : Mortality : In untreated hydrocephalus, death may occur by In untreated hydrocephalus, death may occur by

tonsillar herniation secondary to raised ICP with compression tonsillar herniation secondary to raised ICP with compression of the brain stem and subsequent respiratory arrest.of the brain stem and subsequent respiratory arrest.

Morbidity: Morbidity: 1. Shunt dependence occurs in 75% 1. Shunt dependence occurs in 75% 2. frequent hospitalizations for scheduled shunt revisions .2. frequent hospitalizations for scheduled shunt revisions .3. Poor development of cognitive function in infants and children 3. Poor development of cognitive function in infants and children 4. Visual loss can complicate untreated hydrocephalus and may 4. Visual loss can complicate untreated hydrocephalus and may

persist after treatment persist after treatment

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Male : FemaleMale : Female

Sex: Sex: Generally, incidence is equal in males Generally, incidence is equal in males and females. The exception is and females. The exception is Bickers-Adams Bickers-Adams syndromesyndrome, an X-linked hydrocephalus , an X-linked hydrocephalus transmitted by females and manifested in transmitted by females and manifested in males. NPH has a slight male preponderance. males. NPH has a slight male preponderance.

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Fetal hydrocephalus. Fetal hydrocephalus.

During antenatal care , Fetal ventriculomegaly During antenatal care , Fetal ventriculomegaly can be detected through ultrasound (sonogram) can be detected through ultrasound (sonogram) towards the end of the first trimester. towards the end of the first trimester. Evaluation of the brain and cranial structure is Evaluation of the brain and cranial structure is part of the routine ultrasound examination part of the routine ultrasound examination done by many obstetricians as part of their done by many obstetricians as part of their prenatal care. If the condition is detected on prenatal care. If the condition is detected on ultrasound, the patient may undergo a fetal ultrasound, the patient may undergo a fetal brain MRI (magnetic resonance imaging) to brain MRI (magnetic resonance imaging) to determine the severity of the finding.determine the severity of the finding.

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Sonographic demonstration Sonographic demonstration

Fetal hydrocephalus: Fetal hydrocephalus: gross enlargement of the gross enlargement of the lateral ventricles, lateral ventricles, thinning of the cortex, thinning of the cortex, asymmetric choroid asymmetric choroid plexuses. plexuses.

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InfantsInfants Symptoms Symptoms

Poor feedingPoor feeding IrritabilityIrritability Reduced activityReduced activity VomitingVomiting

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InfantsInfants SignsSigns

Head enlargement: Head circumference is in the 98th percentile for the Head enlargement: Head circumference is in the 98th percentile for the age or greater.(remember h.c. to the age)age or greater.(remember h.c. to the age)

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

veins.(why?)veins.(why?) Tense fontanelle: The anterior fontanelle in infants who are held erect Tense fontanelle: The anterior fontanelle in infants who are held erect

and are not crying may be excessively tense.and are not crying may be excessively tense. Setting-sun sign: In infants it is characteristic of increased ICP. Both Setting-sun sign: In infants it is characteristic of increased ICP. Both

ocular globes are deviated downward, the upper lids are retracted, and ocular globes are deviated downward, the upper lids are retracted, and the white sclerae may be visible above the iris.the white sclerae may be visible above the iris.

Increased limb tone: Spasticity preferentially affects the lower limbs. Increased limb tone: Spasticity preferentially affects the lower limbs. The cause is stretching of the periventricular pyramidal tract fibers by The cause is stretching of the periventricular pyramidal tract fibers by hydrocephalus.hydrocephalus.

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ChildrenChildren Symptoms Symptoms

Slowing of mental capacity Slowing of mental capacity Headaches (initially in the morning) that are more significant than in Headaches (initially in the morning) that are more significant than in

infants because of skull rigidity infants because of skull rigidity Neck pain suggesting tonsillar herniation Neck pain suggesting tonsillar herniation Vomiting, more significant in the morning Vomiting, more significant in the morning Blurred vision - Consequence of papilledema and later of optic atrophy Blurred vision - Consequence of papilledema and later of optic atrophy Double vision - Related to unilateral or bilateral sixth nerve palsy Double vision - Related to unilateral or bilateral sixth nerve palsy Stunted growth and sexual maturation from third ventricle dilatation: This Stunted growth and sexual maturation from third ventricle dilatation: This

can lead to obesity and to precocious or delayed onset of puberty. can lead to obesity and to precocious or delayed onset of puberty. Difficulty in walking secondary to spasticity: This affects the lower limbs Difficulty in walking secondary to spasticity: This affects the lower limbs

preferentially because the periventricular pyramidal tract is stretched by the preferentially because the periventricular pyramidal tract is stretched by the hydrocephalus. hydrocephalus.

DrowsinessDrowsiness

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ChildrenChildrenSignsSigns

Papilledema: if the raised ICP is not treated, this can lead Papilledema: if the raised ICP is not treated, this can lead to optic atrophy and vision loss.to optic atrophy and vision loss.

Failure of upward gaze: This is due to pressure on the tectal Failure of upward gaze: This is due to pressure on the tectal plate through the suprapineal recess.plate through the suprapineal recess.

Macewen sign: A "cracked pot" sound is noted on Macewen sign: A "cracked pot" sound is noted on percussion of the head. Tapping with the fingertips on the percussion of the head. Tapping with the fingertips on the skull skull may show abnormal sounds associated with may show abnormal sounds associated with thinning and separation of skull bones.thinning and separation of skull bones.

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

Large head: Sutures are closed, but chronic increased ICP Large head: Sutures are closed, but chronic increased ICP will lead to progressive abnormal head growth.will lead to progressive abnormal head growth.

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

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Certain picsCertain pics

..

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Certain picsCertain pics

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Differential diagnosisDifferential diagnosis Macrocephaly Macrocephaly

Macrocephaly means a large head—greater than 2 standard Macrocephaly means a large head—greater than 2 standard deviations from the normal distribution; 2% of the “normal” deviations from the normal distribution; 2% of the “normal” population has macrocephaly. Investigation of such population has macrocephaly. Investigation of such individuals may show an abnormality causing macrocephaly, individuals may show an abnormality causing macrocephaly, but many are normal, often with a familial tendency for a large but many are normal, often with a familial tendency for a large head. When asked to evaluate a large head in an otherwise head. When asked to evaluate a large head in an otherwise normal child, first ask the parents for their hat sizes.normal child, first ask the parents for their hat sizes.

The causes of a large head include hydrocephalus (an The causes of a large head include hydrocephalus (an excessive volume of CSF in the skull), megalencephaly excessive volume of CSF in the skull), megalencephaly (enlargement of the brain), thickening of the skull, and (enlargement of the brain), thickening of the skull, and hemorrhage into the subdural or epidural spaces. hemorrhage into the subdural or epidural spaces. Hydrocephalus is the main cause of macrocephaly at birth in Hydrocephalus is the main cause of macrocephaly at birth in which intracranial pressure is increasedwhich intracranial pressure is increased

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Differential diagnosisDifferential diagnosis1- extracranial causes:1- extracranial causes:Cephalhematoma, subgleal hemorrhage .Cephalhematoma, subgleal hemorrhage .2- cranial causes: 2- cranial causes: AnemiaAnemia Cleidocranial dysostosisCleidocranial dysostosis Craniometaphyseal dysplasia of PyleCraniometaphyseal dysplasia of Pyle Epiphyseal dysplasiaEpiphyseal dysplasia HyperphosphatemiaHyperphosphatemia Leontiasis osseaLeontiasis ossea Orodigitofacial dysostosisOrodigitofacial dysostosis Osteogenesis imperfectaOsteogenesis imperfecta OsteopetrosisPyknodysostosisOsteopetrosisPyknodysostosis RicketsRickets Russell dwarfRussell dwarf3-intracranial causes :3-intracranial causes :Megalencephaly , hydrocephalus and strugg – Webber syndrome .Megalencephaly , hydrocephalus and strugg – Webber syndrome .

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Work up Work up

1-Imaging Studies • CT scan of the head • MRI scan of head • Fetal and neonatal cranial ultrasound

2-Diagnostic Procedures • Lumbar puncture

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Work upWork upimaging studiesimaging studies

CT scan of the head delineates the degree of CT scan of the head delineates the degree of ventriculomegaly and, in many cases, the ventriculomegaly and, in many cases, the etiology. When performed with contrast, it can etiology. When performed with contrast, it can show infection and tumors causing show infection and tumors causing obstruction. It also helps with operative obstruction. It also helps with operative planning. Ventricles usually are dilated planning. Ventricles usually are dilated proximal to the point of obstruction. In proximal to the point of obstruction. In pseudotumor cerebri, the CT scan findings pseudotumor cerebri, the CT scan findings usually are normal. usually are normal.

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Work upWork upimaging studiesimaging studies

Perform MRI scan of head in most, if not all, Perform MRI scan of head in most, if not all, congenital cases of hydrocephalus. This congenital cases of hydrocephalus. This delineates the extent of associated brain delineates the extent of associated brain anomalies such as corpus callosum agenesis, anomalies such as corpus callosum agenesis, Chiari malformations, disorders of neuronal Chiari malformations, disorders of neuronal migration, and vascular malformations. migration, and vascular malformations.

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Work upWork upImaging studiesImaging studies

Fetal and neonatal cranial ultrasound is a good Fetal and neonatal cranial ultrasound is a good study for monitoring ventricular size and study for monitoring ventricular size and intraventricular hemorrhage in the neonatal intraventricular hemorrhage in the neonatal ICU setting. Certainly, prior to treatment, ICU setting. Certainly, prior to treatment, perform other imaging studies. perform other imaging studies.

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Imaging studiesImaging studies

Noncommunicating Noncommunicating obstructive hydrocephalus obstructive hydrocephalus caused by obstruction of the caused by obstruction of the foramina of Luschka and foramina of Luschka and Magendie. This MRI Magendie. This MRI sagittal image demonstrates sagittal image demonstrates dilatation of lateral dilatation of lateral ventricles with stretching of ventricles with stretching of corpus callosum and corpus callosum and dilatation of the fourth dilatation of the fourth ventricle. ventricle.

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Noncommunicating Noncommunicating obstructive obstructive hydrocephalus caused hydrocephalus caused by obstruction of by obstruction of foramina of Luschka foramina of Luschka and Magendie. This and Magendie. This MRI axial image MRI axial image demonstrates dilatation demonstrates dilatation of the lateral ventricles. of the lateral ventricles.

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Non-communicating Non-communicating obstructive obstructive hydrocephalus caused hydrocephalus caused by obstruction of by obstruction of foramina of Luschka foramina of Luschka and Magendie. This and Magendie. This MRI axial image MRI axial image demonstrates fourth demonstrates fourth ventricle dilatation. ventricle dilatation.

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Dandy-Walker Dandy-Walker malformation. CT malformation. CT shows cystic dilation of shows cystic dilation of the fourth ventricle and the fourth ventricle and partial agenesis of the partial agenesis of the cerebellar vermis cerebellar vermis

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Aqueductal stenosis. CT Aqueductal stenosis. CT shows marked shows marked enlargement of the third enlargement of the third ventricle (arrow) and ventricle (arrow) and the lateral ventricles.the lateral ventricles.

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Chiari I malformation :Chiari I malformation :

Sagittal C-T1W MR Sagittal C-T1W MR image of the brain image of the brain shows a downward shows a downward herniation of the herniation of the cerebellar tonsils cerebellar tonsils without associated without associated syrinx. syrinx.

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Chiari II malformation :Chiari II malformation :

Axial FSE T2W MR Axial FSE T2W MR image of the brain image of the brain showsshows colpocephaly colpocephaly. .

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MR images demonstrating a massively dilated ventricular system withcongenital hydrocephalus secondary to acqueductal stenosis. The sagittal

image(left) shows a normal-appearing fourth ventricle. The tremendous enlargement of the lateral ventricles has led to compression of the cerebral mantle with only the frontal lobes being visible (right).

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Work upWork upDiagnostic Procedures Diagnostic Procedures

Lumbar puncture can be Lumbar puncture can be used to measure used to measure intracranial pressure, intracranial pressure, but it should only be but it should only be performed after imaging performed after imaging studies rule out an studies rule out an obstruction. Spinal fluid obstruction. Spinal fluid can show the type and can show the type and severity of infection severity of infection ( i.e. in meningitis )( i.e. in meningitis )

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TREATMENT TREATMENT

Medical therapyMedical therapy : : IndicationsIndications: In transient conditions, such as meningitis, or : In transient conditions, such as meningitis, or

neonatal intraventricular hemorrhage .neonatal intraventricular hemorrhage . Modalities :Modalities :

1.1. Acetazolamide (25 mg/kg/d in 3 doses): Careful monitoring Acetazolamide (25 mg/kg/d in 3 doses): Careful monitoring of respiratory status and electrolytes is crucial. Treatment of respiratory status and electrolytes is crucial. Treatment beyond 6 months is not recommended. beyond 6 months is not recommended.

2.2. Furosemide (1 mg/kg/d in 3 doses): Again, electrolyte Furosemide (1 mg/kg/d in 3 doses): Again, electrolyte balance and fluid balance need to be monitored carefully. balance and fluid balance need to be monitored carefully.

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TreatmentTreatment

3-Lumbar punctures: In neonates recovering 3-Lumbar punctures: In neonates recovering from intraventricular hemorrhage, serial from intraventricular hemorrhage, serial lumbar punctures can resolve hydrocephalus lumbar punctures can resolve hydrocephalus in some cases. If possible, this is the in some cases. If possible, this is the preferred method of treatment. preferred method of treatment.

4-Removal of the underlying cause resolves 4-Removal of the underlying cause resolves hydrocephalus in most caseshydrocephalus in most cases

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TreatmentTreatmentSurgical therapySurgical therapy

Principle of surgical intervention :Principle of surgical intervention :

Relief increased ICP by diversion the exessive Relief increased ICP by diversion the exessive CSF from ventricular system into an CSF from ventricular system into an absorptive surface out side the brain such as absorptive surface out side the brain such as pleura or peritoneum or into the atria of the pleura or peritoneum or into the atria of the

heart…….this is called heart…….this is called shunt operationshunt operation The most indication for shunt operation is The most indication for shunt operation is

progressive hydrocephalus .progressive hydrocephalus .

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TreatmentTreatment Contraindications:Contraindications:1.1. The patient in whom a successful surgery would not affect The patient in whom a successful surgery would not affect

the outcome (eg, a child with hydranencephaly) or cortical the outcome (eg, a child with hydranencephaly) or cortical thickenning is less than one cm.thickenning is less than one cm.

2.2. Arrested hydrocephalus is defined as a rare condition in Arrested hydrocephalus is defined as a rare condition in which the neurologic status of the patient is stable in the which the neurologic status of the patient is stable in the presence of stable ventriculomegaly. presence of stable ventriculomegaly.

3.3. Benign hydrocephalus of infancy is found in neonates and Benign hydrocephalus of infancy is found in neonates and young infants. The children are asymptomatic, and head young infants. The children are asymptomatic, and head growth is normal. CT scan shows mildly enlarged ventricles growth is normal. CT scan shows mildly enlarged ventricles and subarachnoid spaces. and subarachnoid spaces.

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TreatmentTreatment

Types of shunts :Types of shunts :1.1. Third ventriculostomy Third ventriculostomy 2.2. Ventriculoperitoneal shunting (the common Ventriculoperitoneal shunting (the common

procedure ) .procedure ) .3.3. Ventriculoatrial shunting .Ventriculoatrial shunting .4.4. Ventriculopleural shunting .Ventriculopleural shunting .5.5. Torkildsen shunts or internal shunts .Torkildsen shunts or internal shunts .6.6. Lumboperitoneal shunts .Lumboperitoneal shunts .

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TreatmentTreatment

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TreatmentTreatment

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Follow-up care Follow-up care Perform CT scan for baseline at 2-4 weeks Perform CT scan for baseline at 2-4 weeks

postsurgery. postsurgery. Monitor all children with shunts every 6-12 months. Monitor all children with shunts every 6-12 months.

Carefully monitor head growth in infants. Check Carefully monitor head growth in infants. Check distal tubing length with plain radiographs when the distal tubing length with plain radiographs when the child grows. Appropriate specialists should carefully child grows. Appropriate specialists should carefully assess child development. assess child development.

What happens to ventricular size in patients who have What happens to ventricular size in patients who have a third ventriculostomy or Torkildsen shunt is not a third ventriculostomy or Torkildsen shunt is not known. Other methods of assessment of patency need known. Other methods of assessment of patency need to be used, such as MRI flow studies and clinical to be used, such as MRI flow studies and clinical evaluations (eg, detailed funduscopic examinations). evaluations (eg, detailed funduscopic examinations).

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COMPLICATIONS COMPLICATIONS

1.1. Infection is the most feared complication Infection is the most feared complication

2.2. Subdural hematomas .Subdural hematomas .

3.3. Shunt failure is mostly due to suboptimal Shunt failure is mostly due to suboptimal proximal catheter placement .proximal catheter placement .

4.4. Overdrainage is more common in Overdrainage is more common in lumboperitoneal shunts .lumboperitoneal shunts .

5.5. Slit ventricle syndrome .Slit ventricle syndrome .

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Shunt failureShunt failure

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PROGNOSIS PROGNOSIS In general, outcome is goodIn general, outcome is good. A typical patient should . A typical patient should

return to baseline after shunting. The neurologic return to baseline after shunting. The neurologic function of children is optimized with shunting. function of children is optimized with shunting.

The best long-term results in the most carefully The best long-term results in the most carefully selected patients are no better than 60% in normal selected patients are no better than 60% in normal pressure hydrocephalus. Few complete recoveries pressure hydrocephalus. Few complete recoveries occur. Often, gait and incontinence respond to occur. Often, gait and incontinence respond to shunting, but dementia responds less frequently. shunting, but dementia responds less frequently.

Often, various other neurologic abnormalities Often, various other neurologic abnormalities associated with hydrocephalus are the limiting factor associated with hydrocephalus are the limiting factor in patient recovery. Examples are migrational in patient recovery. Examples are migrational abnormalities and postinfectious hydrocephalus. abnormalities and postinfectious hydrocephalus.

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Before and AfterBefore and After

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THE ENDTHE END

Health & Medicine

Hydrocephalus project