Hydrocephalus Hydrocephalus Definition-Hydrocephalus is a Definition-Hydrocephalus is a disorder in which the cerebral disorder in which the cerebral ventricular system contains an ventricular system contains an excessive amount of cerebrospinal excessive amount of cerebrospinal fluid (CSF) and is dilated because fluid (CSF) and is dilated because of increased pressure of increased pressure PHYSIOLOGY PHYSIOLOGY CSF is produced - choroid plexus. CSF is produced - choroid plexus. circulates through the circulates through the ventricular system & absorbed into ventricular system & absorbed into the systemic circulation the systemic circulation
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HydrocephalusHydrocephalusDefinition-Hydrocephalus is a disorder in Definition-Hydrocephalus is a disorder in
which the cerebral ventricular system which the cerebral ventricular system contains an excessive amount of contains an excessive amount of
cerebrospinal fluid (CSF) and is dilated cerebrospinal fluid (CSF) and is dilated because of increased pressurebecause of increased pressure
PHYSIOLOGYPHYSIOLOGY
CSF is produced - choroid plexus. CSF is produced - choroid plexus.
circulates through the ventricular circulates through the ventricular system & absorbed into the systemic system & absorbed into the systemic
hydrohydro CSF productionCSF production — — Choroid plexus - located in the cerebral ventricles. Choroid plexus - located in the cerebral ventricles. - consists of villous folds lined by epithelium with a - consists of villous folds lined by epithelium with a
central core of highly vascularized connective tissue. central core of highly vascularized connective tissue. - produces CSF by active secretion and diffusion.- produces CSF by active secretion and diffusion. The production rate of adults is approximately 20 The production rate of adults is approximately 20
mL/hour, turnover 3 – 4x/ d, less in newborns- mL/hour, turnover 3 – 4x/ d, less in newborns- childrenchildren
The volume - infants is = 50 mL & 150 mL adults & The volume - infants is = 50 mL & 150 mL adults & in adults, 25 percent is within the ventricular system.in adults, 25 percent is within the ventricular system.
CSF formation continues in raised intracranial CSF formation continues in raised intracranial pressure unless extremely high pressure unless extremely high
Hydro Hydro Ventricular systemVentricular system — — comprised of lateral ventricles connected via foramen of comprised of lateral ventricles connected via foramen of
Monro to the midline third ventricle.Monro to the midline third ventricle. third ventricle is connected to fourth ventricle aqueduct of third ventricle is connected to fourth ventricle aqueduct of
Sylvius. Sylvius. Three exits from the fourth ventricle, the paired lateral Three exits from the fourth ventricle, the paired lateral
foramina of Luschka and a midline foramen of Magendie, lead foramina of Luschka and a midline foramen of Magendie, lead to a system of interconnecting and focally enlarged areas of to a system of interconnecting and focally enlarged areas of subarachnoid spaces known as cisterns. The cisterns in the subarachnoid spaces known as cisterns. The cisterns in the posterior fossa connect to the subarachnoid spaces over the posterior fossa connect to the subarachnoid spaces over the cerebral convexities through pathways that cross the cerebral convexities through pathways that cross the tentorium. The basal cisterns connect the spinal and tentorium. The basal cisterns connect the spinal and intracranial subarachnoid spaces.intracranial subarachnoid spaces.
CSF absorptionCSF absorption — CSF flows from the lateral ventricles to the — CSF flows from the lateral ventricles to the third and fourth ventricles and then through the basal cisterns, third and fourth ventricles and then through the basal cisterns, tentorium, and subarachnoid space over the cerebral tentorium, and subarachnoid space over the cerebral convexities to the area of the sagittal sinus. The net flow of convexities to the area of the sagittal sinus. The net flow of CSF in the spinal subarachnoid space is cephalad.CSF in the spinal subarachnoid space is cephalad.
CSF is absorbed via arachnoid villi into the venous channels of CSF is absorbed via arachnoid villi into the venous channels of the sagittal sinus. Some CSF absorption also occurs across the the sagittal sinus. Some CSF absorption also occurs across the ependymal lining of the ventricles and from the spinal ependymal lining of the ventricles and from the spinal subarachnoid space.subarachnoid space.
hydrohydro
Hydro Hydro
hydrohydro PATHOGENESISPATHOGENESIS — — imbalance - the production and absorption of CSF. imbalance - the production and absorption of CSF. principal mechanism is deficient absorption= from a principal mechanism is deficient absorption= from a
mechanical or functional obstruction to the flow of CSF= mechanical or functional obstruction to the flow of CSF= excessive volume of CSF = increased ventricular pressure excessive volume of CSF = increased ventricular pressure dilatation.dilatation.
CSF production continues resulting in extreme elevations CSF production continues resulting in extreme elevations of intracranial pressure that preclude neurologic function of intracranial pressure that preclude neurologic function and survival.and survival.
Three mechanisms of imbalance of CSF formation and Three mechanisms of imbalance of CSF formation and absorption: 1.obstruction of CSF pathwaysabsorption: 1.obstruction of CSF pathways
2. impaired venous absorption2. impaired venous absorption 3. oversecretion of CSF. 3. oversecretion of CSF. The disorder that results from obstruction of the ventricular The disorder that results from obstruction of the ventricular
system is known as obstructive, or noncommunicating, system is known as obstructive, or noncommunicating, hydrocephalus Communicating hydrocephalus occurs when hydrocephalus Communicating hydrocephalus occurs when the subarachnoid pathways are blocked. CSF production is the subarachnoid pathways are blocked. CSF production is nearly always normal.nearly always normal.
Hydro Hydro ObstructionObstruction — — Anatomic or functional obstruction - most common Anatomic or functional obstruction - most common
mechanism mechanism The obstruction occurs at the foramen of Monro, the The obstruction occurs at the foramen of Monro, the
aqueduct of Sylvius, or the fourth ventricle and its aqueduct of Sylvius, or the fourth ventricle and its outletsoutlets
Dilatation of the ventricular system occurs proximal to Dilatation of the ventricular system occurs proximal to the block. Obstruction of one foramen of Monro results the block. Obstruction of one foramen of Monro results in dilatation of the lateral ventricle on that side in dilatation of the lateral ventricle on that side
If the aqueduct of Sylvius is blocked- lateral and third If the aqueduct of Sylvius is blocked- lateral and third ventricles dilate, while fourth ventricle remains ventricles dilate, while fourth ventricle remains relatively normal.relatively normal.
Impaired absorptionImpaired absorption — A less common mechanism — A less common mechanism due to inflammation of the subarachnoid villi. This due to inflammation of the subarachnoid villi. This results in communicating hydrocephalus, in which the results in communicating hydrocephalus, in which the entire ventricular system is dilated.entire ventricular system is dilated.
Excessive productionExcessive production — of CSF is a rare cause of — of CSF is a rare cause of hydrocephalus, may occur with a functional choroid hydrocephalus, may occur with a functional choroid plexus papilloma and leads to enlargement of the entire plexus papilloma and leads to enlargement of the entire ventricular system.ventricular system.
pressure and rapid enlargement of the ventricular system. pressure and rapid enlargement of the ventricular system. The frontal and occipital horns of the lateral ventricles The frontal and occipital horns of the lateral ventricles
enlarge first. enlarge first. flattening of gyri & compression of the sulci, obliteration flattening of gyri & compression of the sulci, obliteration
of the subarachnoid of the subarachnoid The vascular system is compressed, & the venous The vascular system is compressed, & the venous
pressure in the dural sinuses increases pressure in the dural sinuses increases Ventricular enlargement =thinning of the cerebral Ventricular enlargement =thinning of the cerebral
mantle& disrupts the ependymal lining = CSF move mantle& disrupts the ependymal lining = CSF move directly into brain tissue = alternate route of CSF directly into brain tissue = alternate route of CSF absorption that may limit further dilatation & contributes absorption that may limit further dilatation & contributes to the development of interstitial edema of the to the development of interstitial edema of the periventricular white matter.periventricular white matter.
compensatory mechanism is spreading of the cranial compensatory mechanism is spreading of the cranial suturessutures
Intracranial pressure is less in chronic hydrocephalus = Intracranial pressure is less in chronic hydrocephalus = the force of is distributed over the greater surface area the force of is distributed over the greater surface area
hydrohydro PATHOLOGYPATHOLOGY — ventricular dilatation before fusion of — ventricular dilatation before fusion of
the cranial sutures = enormous enlargement of the head the cranial sutures = enormous enlargement of the head The intracranial pressure increases more slowlyThe intracranial pressure increases more slowly Marked head enlargement not occur if hydrocephalus Marked head enlargement not occur if hydrocephalus
occurs acutely or after fusion of sutures= significantly occurs acutely or after fusion of sutures= significantly ICPICP
ventricular dilatation results in atrophy of the white ventricular dilatation results in atrophy of the white matter= caused by tissue ischemia from the edema and matter= caused by tissue ischemia from the edema and increased intraventricular pressure. increased intraventricular pressure.
The width of the cerebral mantle may be reduced, gray The width of the cerebral mantle may be reduced, gray matter is better preserved than white matter, even in matter is better preserved than white matter, even in advanced stages.advanced stages.
congenital CNS abnormalities like cortical dysplasias, congenital CNS abnormalities like cortical dysplasias, pachygyria, and polymicrogyria, are associated with X-pachygyria, and polymicrogyria, are associated with X-linked hydrocephalus linked hydrocephalus
Hydro Hydro A.CongenitalA.Congenital — hydrocephalus can result from CNS malformations — hydrocephalus can result from CNS malformations
(which include nonsyndromic and syndromic disorders), infection, (which include nonsyndromic and syndromic disorders), infection, trauma, and teratogens- rare cause is obstruction by a congenital CNS trauma, and teratogens- rare cause is obstruction by a congenital CNS tumor, located near the midline.tumor, located near the midline.
Neural tube defectsNeural tube defects — majority with myelomeningocele have — majority with myelomeningocele have hydrocephalus. =obstruction of fourth ventricular outflow or flow of hydrocephalus. =obstruction of fourth ventricular outflow or flow of CSF through the posterior fossa due to the Chiari malformation or an CSF through the posterior fossa due to the Chiari malformation or an associated aqueductal stenosis.associated aqueductal stenosis.
Isolated hydrocephalusIsolated hydrocephalus — by aqueductal stenosis due to congenital — by aqueductal stenosis due to congenital narrowing of the aqueduct, or intrauterine infection.narrowing of the aqueduct, or intrauterine infection.
X-linked hydrocephalusX-linked hydrocephalus — most common genetic form of congenital — most common genetic form of congenital hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS)]. hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS)]. Approximately 50% affected boys have adducted thumbs. Some have Approximately 50% affected boys have adducted thumbs. Some have other CNS abnormalities such as agenesis or dysgenesis of the corpus other CNS abnormalities such as agenesis or dysgenesis of the corpus callosum, small brainstem, or absence of the pyramidal tract.callosum, small brainstem, or absence of the pyramidal tract.
is due to mutations in the gene encoding L1, a neuronal cell adhesion is due to mutations in the gene encoding L1, a neuronal cell adhesion molecule that belongs to the immunoglobulin superfamily and is molecule that belongs to the immunoglobulin superfamily and is essential in neurodevelopmen. The gene for L1 has been mapped to essential in neurodevelopmen. The gene for L1 has been mapped to Xq28. Mutations in L1 also result in other conditions, known as the L1 Xq28. Mutations in L1 also result in other conditions, known as the L1 spectrum, that are characterized by neurologic abnormalities and spectrum, that are characterized by neurologic abnormalities and mental retardation. These include MASA spectrum (Mental retardation, mental retardation. These include MASA spectrum (Mental retardation, Aphasia, Shuffling gait, Adducted thumbs), X-linked spastic paraplegia Aphasia, Shuffling gait, Adducted thumbs), X-linked spastic paraplegia type 1, and X-linked agenesis of the corpus callosum.type 1, and X-linked agenesis of the corpus callosum.
CNS malformationsCNS malformations — frequently associated with hydrocephalus. — frequently associated with hydrocephalus. Chiari malformation, accompanies a neural tube defect, the brain stem Chiari malformation, accompanies a neural tube defect, the brain stem
and cerebellum- displaced caudally obstructing the flow of CSF in the and cerebellum- displaced caudally obstructing the flow of CSF in the posterior fossa posterior fossa
The Dandy-Walker malformation consists of a large posterior fossa cyst The Dandy-Walker malformation consists of a large posterior fossa cyst continuous with the fourth ventricle and defective development of the continuous with the fourth ventricle and defective development of the cerebellum, including partial or complete absence of the vermis cerebellum, including partial or complete absence of the vermis =secondary obstruction of the foramina of Luschka and Magendie. =secondary obstruction of the foramina of Luschka and Magendie.
Vein of Galen malformation is a rare cause = compression of the Vein of Galen malformation is a rare cause = compression of the aqueduct of Sylvius by the markedly dilated and distorted vein . aqueduct of Sylvius by the markedly dilated and distorted vein .
Syndromic formsSyndromic forms - The most frequent are trisomies 13, 18, 9 and 9p, - The most frequent are trisomies 13, 18, 9 and 9p, and triploidy and triploidy
Intrauterine infectionIntrauterine infection -rubella, cytomegalovirus, toxoplasmosis, and -rubella, cytomegalovirus, toxoplasmosis, and syphilis = inflammation of the ependymal lining of the ventricular syphilis = inflammation of the ependymal lining of the ventricular system and the meninges in the subarachnoid space = obstruction of system and the meninges in the subarachnoid space = obstruction of CSF flow through the aqueduct or basal cisterns.CSF flow through the aqueduct or basal cisterns.
Acquired hydrocephalusAcquired hydrocephalus — Common are CNS infections - bacterial — Common are CNS infections - bacterial meningitis or viral infections including mumps, and tumors, especially meningitis or viral infections including mumps, and tumors, especially posterior fossa medulloblastomas, astrocytomas, and ependymomas. posterior fossa medulloblastomas, astrocytomas, and ependymomas.
Another cause is hemorrhage into the subarachnoid space or, less Another cause is hemorrhage into the subarachnoid space or, less commonly, into the ventricular system, by ruptured aneurysms, commonly, into the ventricular system, by ruptured aneurysms, arteriovenous malformations, trauma, or systemic bleeding disorders= arteriovenous malformations, trauma, or systemic bleeding disorders= induces an inflammatory response followed by fibrosis, obstructing the induces an inflammatory response followed by fibrosis, obstructing the flow and/or absorption of CSF.flow and/or absorption of CSF.
Posthemorrhagic hydrocephalus occurs in approximately 35 percent of Posthemorrhagic hydrocephalus occurs in approximately 35 percent of preterm infants with intraventricular hemorrhage (IVH). It can be preterm infants with intraventricular hemorrhage (IVH). It can be obstructive, communicating, or both, and can be transient or sustained, obstructive, communicating, or both, and can be transient or sustained, with slow or rapid progression. with slow or rapid progression.
Hydro Hydro CLINICAL FEATURESCLINICAL FEATURES — signs and symptoms result from: — signs and symptoms result from: - increased ICP and dilatation of the ventricles - increased ICP and dilatation of the ventricles -time of presentation depends upon the acuity of the process. -time of presentation depends upon the acuity of the process. Symptoms are nonspecific and independent of the etiology Symptoms are nonspecific and independent of the etiology Headache is a prominent symptom. It is caused by distortion Headache is a prominent symptom. It is caused by distortion
of the meninges and blood vessels. The pain often varies in of the meninges and blood vessels. The pain often varies in intensity and location and may be intermittent or persistent. intensity and location and may be intermittent or persistent. Headaches due to increased ICP often occur in the early Headaches due to increased ICP often occur in the early morning and are associated with nausea and vomitingmorning and are associated with nausea and vomiting
often have changes in their personality and behavior often have changes in their personality and behavior (irritability, obstreperousness, indifference, and loss of (irritability, obstreperousness, indifference, and loss of interest), mechanism is uncertain, but related in part to interest), mechanism is uncertain, but related in part to increased ICP. As the hydrocephalus worsens, midbrain and increased ICP. As the hydrocephalus worsens, midbrain and brain stem dysfunction may result in lethargy and drowsiness. brain stem dysfunction may result in lethargy and drowsiness.
Increased ICP in the posterior fossa often leads to nausea, Increased ICP in the posterior fossa often leads to nausea, vomiting, and decreased appetite.vomiting, and decreased appetite.
Hydro Hydro
In infants, common signs and symptoms of In infants, common signs and symptoms of hydrocephalus include:hydrocephalus include:
An unusually large head An unusually large head A rapid increase in the size of the head A rapid increase in the size of the head A bulging "soft spot" on the top of the A bulging "soft spot" on the top of the
head (anterior fontanel) head (anterior fontanel) Vomiting Vomiting Sleepiness Sleepiness Irritability Irritability Seizures Seizures Eyes fixed downward (sunsetting of the Eyes fixed downward (sunsetting of the
In older children and adults, common signs and In older children and adults, common signs and symptoms of hydrocephalus include:symptoms of hydrocephalus include:
Headache followed by vomiting Headache followed by vomiting Nausea Nausea Blurred or double vision Blurred or double vision Eyes fixed downward (sunsetting of the eyes) Eyes fixed downward (sunsetting of the eyes) Problems with balance, coordination or gait Problems with balance, coordination or gait Sluggishness or lack of energy Sluggishness or lack of energy Slowed development or loss of development Slowed development or loss of development Memory loss Memory loss Urinary incontinence Urinary incontinence Irritability Irritability Change in personalityChange in personality
HydroHydro Physical examinationPhysical examination — Physical findings are due to the effects — Physical findings are due to the effects
ICP. ICP. Distortions of the brainstem may result in changes in vital signs such Distortions of the brainstem may result in changes in vital signs such
as bradycardia, systemic hypertension, and altered respiratory rate. as bradycardia, systemic hypertension, and altered respiratory rate. Excessive head growth & ventricular dilatation occur before head Excessive head growth & ventricular dilatation occur before head
growth becomes abnormal. growth becomes abnormal. The anterior fontanelle may become full or distended. The anterior fontanelle may become full or distended. Young infants may develop frontal bossing, an abnormal skull Young infants may develop frontal bossing, an abnormal skull
contour in which the forehead becomes prominent. contour in which the forehead becomes prominent. The scalp veins may appear dilated and prominent. The scalp veins may appear dilated and prominent. Compression of the third or sixth cranial nerve may result in Compression of the third or sixth cranial nerve may result in
extraocular muscle pareses leading to diplopia. Pressure on the extraocular muscle pareses leading to diplopia. Pressure on the midbrain may result in impairment of upward gaze. This is known as midbrain may result in impairment of upward gaze. This is known as the setting-sun sign because of the appearance of the sclera visible the setting-sun sign because of the appearance of the sclera visible above the iris. above the iris.
Fundoscopic examination may reveal papilledema. Fundoscopic examination may reveal papilledema. Stretching of the fibers from the motor cortex around the dilated Stretching of the fibers from the motor cortex around the dilated
ventricles may result in spasticity of the extremities, especially the ventricles may result in spasticity of the extremities, especially the legs. legs.
Accelerated pubertal development, as well as disturbed growth and Accelerated pubertal development, as well as disturbed growth and fluid and electrolyte homeostasis, may result from pressure of the fluid and electrolyte homeostasis, may result from pressure of the dilated third ventricle on the hypothalamus dilated third ventricle on the hypothalamus
hydrohydro DIAGNOSISDIAGNOSIS — suspect in an infant whose head — suspect in an infant whose head
circumference is enlarged at birth, or when serial circumference is enlarged at birth, or when serial measurements cross growth curves, measurements cross growth curves,
In some cases, the diagnosis is made by antenatal In some cases, the diagnosis is made by antenatal ultrasonography. Hydrocephalus should be considered in ultrasonography. Hydrocephalus should be considered in children with severe headache and other features suggesting children with severe headache and other features suggesting increased ICPincreased ICP
The diagnosis is confirmed by neuroimaging. The diagnosis is confirmed by neuroimaging. In older infants and children, CT or MRI should be performed. In older infants and children, CT or MRI should be performed.
Neuroimaging studies will also detect associated CNS Neuroimaging studies will also detect associated CNS malformations or tumors.malformations or tumors.
The site of obstructed CSF flow may be suggested by the The site of obstructed CSF flow may be suggested by the pattern of ventricular dilatation. Stenosis of the aqueduct pattern of ventricular dilatation. Stenosis of the aqueduct typically results in dilated lateral and third ventricles and a typically results in dilated lateral and third ventricles and a fourth ventricle of normal size. In contrast, an extraventricular fourth ventricle of normal size. In contrast, an extraventricular obstruction usually results in symmetric dilatation of all obstruction usually results in symmetric dilatation of all ventricles.ventricles.
A lumbar puncture (LP) should be performed and the CSF A lumbar puncture (LP) should be performed and the CSF should be examined if an infection causing adhesive should be examined if an infection causing adhesive arachnoiditis or ependymitis is suspected. However, LP is arachnoiditis or ependymitis is suspected. However, LP is contraindicated if the patient has evidence of a space-contraindicated if the patient has evidence of a space-occupying lesion such as an intracranial tumor or a brain occupying lesion such as an intracranial tumor or a brain abscess, because of the risk of cerebral herniation.abscess, because of the risk of cerebral herniation.
hydrohydro MANAGEMENTMANAGEMENT — The most effective is — The most effective is
surgical drainage- does not cure but treat the surgical drainage- does not cure but treat the symptoms and stops progression.symptoms and stops progression.
ShuntShunt — A mechanical shunt system involves — A mechanical shunt system involves placement of a catheter into one of the lateral placement of a catheter into one of the lateral ventricles, usually the rightventricles, usually the right
The catheter is connected to a one-way valve The catheter is connected to a one-way valve system (usually placed beneath the scalp of system (usually placed beneath the scalp of the postauricular area) that opens when the the postauricular area) that opens when the pressure in the ventricle exceeds a certain pressure in the ventricle exceeds a certain valuevalue
ventriculoatrial, VA or ventriculoperitoneal, VPventriculoatrial, VA or ventriculoperitoneal, VP
HydroHydro -Fig .VP & VA shunts -Fig .VP & VA shunts
hydrohydro
ComplicationsComplications The severity of hydrocephalus depends on the time The severity of hydrocephalus depends on the time
of onset and whether the disease is progressive. If of onset and whether the disease is progressive. If the condition is well advanced at birth, major brain the condition is well advanced at birth, major brain damage and physical disabilities are likely. In less damage and physical disabilities are likely. In less severe cases, with proper treatment, it's possible to severe cases, with proper treatment, it's possible to have a nearly normal life span and intelligence.have a nearly normal life span and intelligence.
Other complications of hydrocephalus include:Other complications of hydrocephalus include: Intellectual impairment Intellectual impairment Neurological damage, such as decreased function, Neurological damage, such as decreased function,
movement or sensation movement or sensation Problems with the artificial CSF drainage channel Problems with the artificial CSF drainage channel
(surgical shunt), such as a blockage or kinking of the (surgical shunt), such as a blockage or kinking of the shunt tubing shunt tubing
Infection at the site of the shunt Infection at the site of the shunt
hydrohydro ComplicationsComplications — In general, complications of — In general, complications of
treated hydrocephalus are due to malfunction treated hydrocephalus are due to malfunction of the shunt. If the hydrocephalus is still active, of the shunt. If the hydrocephalus is still active, symptoms recur and another drainage symptoms recur and another drainage procedure is required. Shunt revision is procedure is required. Shunt revision is unnecessary in rare cases when alternate unnecessary in rare cases when alternate pathways of absorption develop or normal pathways of absorption develop or normal mechanisms for handling CSF become mechanisms for handling CSF become reestablished, resulting in compensation or reestablished, resulting in compensation or spontaneous arrest of the hydrocephalus.spontaneous arrest of the hydrocephalus.
Malfunction is due to infection or mechanical Malfunction is due to infection or mechanical failure Approximately 40 percent of standard failure Approximately 40 percent of standard shunts fail within the first year after placement .shunts fail within the first year after placement .
Hydro Hydro InfectionInfection — Shunt infection is common = 5 to 10 percent of — Shunt infection is common = 5 to 10 percent of
procedures . may be higher in newborns . Most occur in the first procedures . may be higher in newborns . Most occur in the first six monthst. Ventriculitis may develop.six monthst. Ventriculitis may develop.
organisms are own skin flora, such as Staphylococcus epidermidis, organisms are own skin flora, such as Staphylococcus epidermidis, less frequently S. aureus, enteric bacteria, diphtheroids, and less frequently S. aureus, enteric bacteria, diphtheroids, and Streptococcus species .Streptococcus species .
Infection must be considered with a shunt & persistent fever. Infection must be considered with a shunt & persistent fever. Antibiotics should be started, but alone is usually not effective. In Antibiotics should be started, but alone is usually not effective. In most cases, an infected shunt must be removed and an external most cases, an infected shunt must be removed and an external ventricular drain temporarily placed.ventricular drain temporarily placed.
Shunt infections may promote the development of loculated Shunt infections may promote the development of loculated compartments of CSF and contribute to impaired cognitive compartments of CSF and contribute to impaired cognitive outcome and death . Perioperative antibiotic prophylaxis in a outcome and death . Perioperative antibiotic prophylaxis in a meta-analysis of 12 trials in 1359 patients, reduced the risk of meta-analysis of 12 trials in 1359 patients, reduced the risk of subsequent shunt infection by 50 percent.subsequent shunt infection by 50 percent.
Mechanical failureMechanical failure — The failure rate (including infection) is — The failure rate (including infection) is approximately 40 percent in the first year, and 5 percent in approximately 40 percent in the first year, and 5 percent in subsequent years .subsequent years .
The majority of first shunt failures result from obstruction at the The majority of first shunt failures result from obstruction at the ventricular catheter. This is because shunts typically overdrain, ventricular catheter. This is because shunts typically overdrain, greatly reducing the size of the ventricles=catheter to lie against greatly reducing the size of the ventricles=catheter to lie against the ependyma and choroid plexus which block the holes at the end the ependyma and choroid plexus which block the holes at the end of the catheter.of the catheter.
Fractured tubing is the cause in 15 percent of cases . Other causes Fractured tubing is the cause in 15 percent of cases . Other causes include migration of part or all of the shunt (7.5 percent) and include migration of part or all of the shunt (7.5 percent) and problems with overdrainage (7 percent).problems with overdrainage (7 percent).
Hydro Hydro Third ventriculostomyThird ventriculostomy — Endoscopic third ventriculostomy — Endoscopic third ventriculostomy
(ETV), perforation is made to connect the third ventricle to the (ETV), perforation is made to connect the third ventricle to the subarachnoid space, in the initial treatment of selected cases of subarachnoid space, in the initial treatment of selected cases of obstructive hydrocephalus and as an alternative to shunt revision. obstructive hydrocephalus and as an alternative to shunt revision. The success depends upon the cause and previous complications The success depends upon the cause and previous complications
Medical therapyMedical therapy — diuretics, fibrinolysis, and serial lumbar — diuretics, fibrinolysis, and serial lumbar punctures- have significant complications and are less effective punctures- have significant complications and are less effective than surgical treatment.than surgical treatment.
DiureticsDiuretics — The diuretics — The diuretics furosemidefurosemide and and acetazolamideacetazolamide decrease CSF production. They have been used for short periods decrease CSF production. They have been used for short periods in slowly progressive hydrocephalus in patients too unstable for in slowly progressive hydrocephalus in patients too unstable for surgery. Diuretics are also used in newborns with posthemorrhagic surgery. Diuretics are also used in newborns with posthemorrhagic hydrocephalus, although their use is controversial .hydrocephalus, although their use is controversial .
A systematic review by the Cochrane database examined two A systematic review by the Cochrane database examined two eligible trials of diuretic therapy in newborns . These trials eligible trials of diuretic therapy in newborns . These trials included 177 and 16 infants with posthemorrhagic ventricular included 177 and 16 infants with posthemorrhagic ventricular dilation. The administration of dilation. The administration of acetazolamideacetazolamide and and furosemidefurosemide did did not decrease the risk for VP shunt or the combined outcome of not decrease the risk for VP shunt or the combined outcome of shunt or death. In the larger trial, the administration of shunt or death. In the larger trial, the administration of acetazolamide and furosemide was associated with a borderline acetazolamide and furosemide was associated with a borderline increase in the risk for motor impairment at one year (relative risk increase in the risk for motor impairment at one year (relative risk 1.27) . However, the combined outcome of delay, disability or 1.27) . However, the combined outcome of delay, disability or motor impairment among survivors, or the risk of the combined motor impairment among survivors, or the risk of the combined outcome of death, delay, disability or impairment at one year outcome of death, delay, disability or impairment at one year were not affected. Diuretic treatment significantly increased the were not affected. Diuretic treatment significantly increased the risk of nephrocalcinosis.risk of nephrocalcinosis.
administration of fibrinolytic agents has been administration of fibrinolytic agents has been used in newborns with posthemorrhagic used in newborns with posthemorrhagic hydrocephalus in an attempt to prevent hydrocephalus in an attempt to prevent permanent obstruction to CSF flow. This permanent obstruction to CSF flow. This treatment does not appear to reduce the need for treatment does not appear to reduce the need for shunt placement and may increase the risk of shunt placement and may increase the risk of hemorrhage, but adequate trials are lacking hemorrhage, but adequate trials are lacking
Serial lumbar puncturesSerial lumbar punctures — Repeated lumbar — Repeated lumbar punctures - as a temporizing measure in preterm punctures - as a temporizing measure in preterm infants with posthemorrhagic hydrocephalusinfants with posthemorrhagic hydrocephalus
In cases of rapidly progressive hydrocephalus, a In cases of rapidly progressive hydrocephalus, a temporary ventricular drainage device may be temporary ventricular drainage device may be needed until a permanent shunt can be placed or needed until a permanent shunt can be placed or the hydrocephalus resolves spontaneously the hydrocephalus resolves spontaneously
Hydro Hydro OUTCOMEOUTCOME — depends upon the etiology, associated abnormalities, and — depends upon the etiology, associated abnormalities, and
complications such as infection.complications such as infection. SurvivalSurvival — untreated -50 percent die before three years and 77 to 80 — untreated -50 percent die before three years and 77 to 80
percent die before reaching adulthood Treatment( if no tumor) with 89 percent die before reaching adulthood Treatment( if no tumor) with 89 and 95 percent survivaland 95 percent survival
EpilepsyEpilepsy — occur frequently in children with shunted hydrocephalus. — occur frequently in children with shunted hydrocephalus. Seizures are associated with poor cognitive outcomeSeizures are associated with poor cognitive outcome Functional outcomeFunctional outcome — depends upon prematurity, CNS malformations, — depends upon prematurity, CNS malformations,
other congenital abnormalities, epilepsy, and sensory and motor other congenital abnormalities, epilepsy, and sensory and motor impairments impairments
In a report from France, outcome at 10 years was evaluated in 129 In a report from France, outcome at 10 years was evaluated in 129 consecutive children with hydrocephalus without tumor who had shunt consecutive children with hydrocephalus without tumor who had shunt placement before two years of age . Motor deficits, visual or auditory placement before two years of age . Motor deficits, visual or auditory deficits, and epilepsy occurred in 60, 25, and 30 percent of patients, deficits, and epilepsy occurred in 60, 25, and 30 percent of patients, respectively. IQ was >90 in 32 percent and <50 in 21 percent. Attendance respectively. IQ was >90 in 32 percent and <50 in 21 percent. Attendance at a normal school was possible for 60 percent, although one-half were at a normal school was possible for 60 percent, although one-half were one to two years behind for their age or having difficulties. Of the one to two years behind for their age or having difficulties. Of the remainder, 31 percent were in special classes or institutionalized and 9 remainder, 31 percent were in special classes or institutionalized and 9 percent were not considered educable. percent were not considered educable.
In a series from the United Kingdom, 155 children with shunted In a series from the United Kingdom, 155 children with shunted hydrocephalus were followed for 10 years or until death (which occurred hydrocephalus were followed for 10 years or until death (which occurred in 11 percent) . For survivors until school age, 59 percent attended a in 11 percent) . For survivors until school age, 59 percent attended a normal school. Children with hydrocephalus caused by infection or IVH normal school. Children with hydrocephalus caused by infection or IVH were more likely to need special school than those with congenital were more likely to need special school than those with congenital hydrocephalus (52 and 60 versus 29 percent). hydrocephalus (52 and 60 versus 29 percent).
Case historyCase history Age 15yr/M sudent, MersaAge 15yr/M sudent, Mersa C/C behavioral change/1&1/2 yearsC/C behavioral change/1&1/2 years Irritable, over talkative, aggression Irritable, over talkative, aggression
asaultive, grandiose, crying asaultive, grandiose, crying Headache, vomiting, weakness, sweating Headache, vomiting, weakness, sweating Repeated attacks of ear dischargeRepeated attacks of ear discharge Failure to thrive Failure to thrive Got worse 8-months back-scaly lesions Got worse 8-months back-scaly lesions
complaintcomplaint Recent enuresis but no incontinenceRecent enuresis but no incontinence Negative symptomsNegative symptoms
History contdHistory contd
Development –non remarkableDevelopment –non remarkable He used to be calm & good studentHe used to be calm & good student Family-father died cough & Family-father died cough &
empyemia-9monempyemia-9mon Mother on ART, 4-other siblings-one Mother on ART, 4-other siblings-one
younger sister healthyyounger sister healthy
Physical findingPhysical finding
GA- Chronically sick GA- Chronically sick V/S-B/P __ P/R=78, T=36.7V/S-B/P __ P/R=78, T=36.7 scaly multiple lesions over scalp, LAPscaly multiple lesions over scalp, LAP Labile mood, appropriate affect, has Labile mood, appropriate affect, has
