chapter 1,2,4 full case pediatric hidrocephalus
TRANSCRIPT
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CHAPTER 1
INTRODUCTION
The word "hydrocephalus" is derived from two Greek words: hydro meaning
water; and cephalus meaning head; also known as "water on the brain". Historically it
is believed to result from imbalance between CSF production and absorption, with
net accumulation of fluid in the cranial cavity; characterized by increase in size of the
cerebral ventricles. It is classified as: Communicating hydrocephalus, in which flow
is not obstructed, but CSF is inadequately reabsorbed in the subarachnoid space and
the Non-communicating hydrocephalus or the Obstructive type, in which flow of CSF
from the ventricles to subarachnoid space is obstructed. This type may also be sub-
classified into Congenital and Acquired. The overall incidence of hydrocephalus is
not known. Approximately 55% of all hydrocephalus are congenital.1,2,3
The etiology depends upon the age of the child. The clinical features are
increase in the size of head, with wide anterior fontanelle, prominent scalp veins, sun-
setting eyes, optic nerve atrophy, nystagmus and increased muscle tone in children
upto 2 years. Children more than 2 years may present with these as hydrocephalus
progresses; or if the fontanelles are closed, head size may be normal. These may
present with optic atrophy or papilloedema, abnormal hypothalamic functions (short
stature or gigantism, obesity, delayed puberty, primary amenorrhea or menstrual
irregularity and diabetes inspidus) and spastic lower limbs.3,4
Performance IQ is worse than verbal IQ and learning problems are common.
The diagnostic procedures include measurement of head circumference, Plain X ray
of head, Ventriculography, Pneumoencephalography, Ultrasonography, Computed
Tomography and Magnetic Resonance Imaging. The management may be non-
surgical and surgical.3,4
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CHAPTER 2
REVIEW OF RELATED LITERATURE
2.1. Definition1,2,3
The word hydrocephalus comes from the Greek hydro, meaning water, and
cephalie,meaning the brain, so it literally means water on the brain. Hydrocephalus
is a condition in which excess fluid accumulates in the brain. It is often referred to as
"water on the brain," the "water" is actually cerebrospinal fluid (CSF) -- a clear fluid
surrounding the brain and spinal cord.
Hydrocephalus can be defined broadly as a disturbance of cerebrospinal fluid
(CSF) formation, flow, or absorption, leading to an increase in volume occupied by
this fluid in the central nervous system (CNS). This condition could also be termed a
hydrodynamic CSF disorder. Hydrocephalus is characterized by an increased volume
of CSF in association with progressive ventricular dilation
2.2. Etiology2,3,4
Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is
present at birth and may be caused by either environmental influences during fetal
development or genetic predisposition. Acquired hydrocephalus develops at the time
of birth or at some point afterward. This type of hydrocephalus can affect individuals
of all ages and may be caused by injury or disease.
The etiology differs in age group up to 2 and beyond 2 years. Table 2.1.
presents etiologies according to age groups.12-14
0 to 2 Years More than 2 to 12 years
Intra uterine
infections
AnoxicBacterial,
granulomatous,parasitic
Mass lesion
compressing
the ventricular
Craniopharyngioma Pineal tumor
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Traumatic perinatalheamorrhage
system
Neonatal, bacterial or viralmeningio-encephalitis
Posteriorfossa tumor
MedulloblastomaAstrocytoma
Ependymona
Arachnoid cyst Deveplomenta
l disorders
Aqueductal stenosisArnold-chiari malformation
Intracranial tumours Post
infectious
Meningitis (bacterial
and granulamatous)
Arterivenous malformation of the
galenic system
Post haemorrhagic
Post infectious (bacterial and
granulomatous meningitis)
Developmen
t disorders
Aqueductalstenosis
Myelomeningiocoele
Dandy-walker cyst Encephalocoele
Table 2.1. etiologies according to age groups3
Hydrocephalus may also be communicating or non-communicating.
Communicating hydrocephalus occurs when the flow of CSF is blocked after it exits
from the ventricles. This form is called communicating because the CSF can still flow
between the ventricles, which remain open. Noncommunicating hydrocephalus - also
called "obstructive" hydrocephalus - occurs when the flow of CSF is blocked along
one or more of the narrow pathways connecting the ventricles. One of the most
common causes of hydrocephalus is "aqueductal stenosis." In this case,
hydrocephalus results from a narrowing of the aqueduct of Sylvius, a small
passageway between the third and fourth ventricles in the middle of the brain.
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There are two other forms of hydrocephalus which do not fit distinctly into
the categories mentioned above and primarily affect adults: hydrocephalus ex-vacuo
and normal pressure hydrocephalus. Hydrocephalus ex-vacuo occurs when there is
damage to the brain caused by stroke or traumatic injury. In these cases, there may be
actual shrinkage (atrophy or wasting) of brain tissue. Normal pressure hydrocephalus
can occur in people of any age, but it is most common in the elderly population. It
may result from a subarachnoid hemorrhage, head trauma, infection, tumor, or
complications of surgery. However, many people develop normal pressure
hydrocephalus even when none of these factors are present. In these cases the cause
of the disorder is unknown.
The causes of hydrocephalus are not all well understood. Hydrocephalus may
result from genetic inheritance (aqueductal stenosis) or developmental disorders such
as those associated with neural tube defects including spina bifida and encephalocele.
Other possible causes include complications of premature birth such as
intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head
injury, or subarachnoid hemorrhage.
Premature infants have an increased risk of intraventricular hemorrhage in
which severe bleeding ithin the ventricles of the brain can lead to hydrocephalus.
Other problems that can occur during pregnancy may increase an infant's risk of
developing hydrocephalus, including intrauterine infection or a disorder involving
incomplete closure of an infant's spinal column (myelomeningocele). Congenital or
developmental defects can increase older children's risk of hydrocephalus. Lesions or
tumors of the brain or spinal cord, central nervous system infections, bleeding in the
brain, and severe head injury also can increase the risk of hydrocephalus.
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Figure 2.1. Causes of Hydrocephalus5
2.3. Incidence1,3,5
Incidence and prevalence data are difficult to establish as there is no existing
national registry or database of people with hydrocephalus and closely associated
disorders; however, hydrocephalus is believed to affect approximately 1 in every 500
children. At present, most of these cases are diagnosed prenatally, at the time of
delivery, or in early childhood. Advances in diagnostic imaging technology allow
more accurate diagnoses in individuals with atypical presentations, including adults
with conditions such as normal pressure hydrocephalus.
In the United States, a little over 1 in 1000 births are affected byhydrocephalus.
Hydrocephalus is one of the most common "birth defects" and afflicts inexcess of 10,000 babies each year.
Studies by the World Health Organization show that one birth in every 2,000result in hydrocephalus.
There are 70,000 discharges a year from hospitals in the United States with adiagnosis of hydrocephalus.
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More than 50% of hydrocephalus cases are congenital. As many as 75% of children with hydrocephalus will have some form of
motor disability.
Over the past 25 years, death rates associated with hydrocephalus havedecreased from 54% to 5%; intellectual disabilitity has decreased from 62% to
30%.
About 80% of hydrocephalus patients are born with other defects. Othermedical conditions usually associated with Hydrocephalus include:
o Arachnoid Cysts.o Brain Injury.o Dandy-Walker Syndrome.o Head Trauma.o Meninigitis.o Porencephaly.o Tumors.o Spina Bifida.
Hydrocephalus occurs in 70 to 90% of children with the most severe form ofSpina Bifida.
2.4. Pathophysiology3,5,6
Pathophysiologically, hydrocephalus is regarded as an imbalance in the
formation and absorption of cerebrospinal fluid (CSF) to a sufficient magnitude
producing accumulation of fluid leading to an elevation of intracranial pressure.
Compensatory adjustments especially in very young and very old subjects may occur
that may reduce prevailing CSF pressure to normal range. Thus, hydrocephalus must
be carefully distinguished from cerebral atrophy, in which an excessive accumulation
of CSF within the intracranial cavities is due to loss of cerebral substance rather than
a primary defect in CSF formation or absorption.
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Figure 2.1. CSF Circulation and Ventricular Sistem6
Cerebrospinal Fluid (CSF) formation3
CSF is mainly formed within the ventricular system. Formation sites include choroid
plexus, ependyma and parenchyma. Most of the CSF is formed by the choroids
plexus of lateral ventricles. The rate of formation is 0.35-0.40 ml/min equivalent to
500 ml per day, the rate being same in paediatric and adult population.
CSF Pathways3
Lateral Ventricles Intraventricular foramen of Monro 3rd Ventricle
Aqueduct of Sylivus 4th
Ventricle Foramen of Luska and Magendie
subarachnoid space and Spinal Canal.
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Figure 2.2. CSF Formation7
Figure 2.3. CSF Circulatory Pathway6
The large head attributed to water on the brain has long attracted interest andspeculation. Hypothetically, hydrocephalus can be subdivided into 3 forms:
Disorders of cerebrospinal fluid production: This is the rarest form ofhydrocephalus. Choroid plexus papillomas and choroid plexus carcinomas can
secrete cerebrospinal fluid in excess of its absorption.
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Disorders of cerebrospinal fluid circulation: This form of hydrocephalusresults from obstruction of the pathways of cerebrospinal fluid circulation.
This can occur at the ventricles or arachnoid villi. Tumors, hemorrhages,
congenital malformations, and infections can cause obstruction at either point
in the pathways.
Disorders of cerebrospinal fluid absorption: Conditions, such as the superiorvena cava syndrome and sinus thrombosis, can interfere with cerebrospinal
fluid absorption.
Some forms of hydrocephalus cannot be classified clearly. This group includes
normal pressure hydrocephalus and pseudotumor cerebri.
2.5. Classification2,3,8,
In the very ancient times, Hippocrates recognized accumulation of water in
the head and the cavities of brain as the cause of enlarged head. Two hundred years
later, Magendie and Lusckha described the communication between ventricular
system and subarachnoid spaces. Later, Key and Retzuis in 1872, Dandy and
Blackfen in 1913 and 1914 and Weed in 1920, demonstrated that this can result due
to obstruction of ventricular system. Thus, hydrocephalus was divided into two types:
Obstructive due to blockage; and Communicating secondary to decreased absorption
over the surface of brain.
The classification being used presently is as follows:
1. Non-communicating or obstructive hydrocephalus in which flow of CSF fromthe ventricles to subarachnoid space is obstructed. Thus there is no
communication between the ventricular system and the subarachnoid space.
The commonest cause of this category is aqueduct blockage.
2. Communicating or non-obstructive hydrocephalus in which flow is notobstructed, but CSF is inadequately reabsorbed in the subarachnoid space.
Thus there is communication between the ventricular system and the
subarachnoid space. The commonest cause of this group is post-infective and
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post-haemorrhagic hydrocephalus. This type may also be sub-classified into
Congenital and Acquired. Approximately 55% of all hydrocephalus are
congenital. The reported incidence of primarily congenital hydrocephalus is
0.9 to 1.5 per 1000 births and those occurring with spina bifida and
myelomeningocele varies from 1.3 to 2.9 per 1000 per births.
2.6.Clinical features3,8,9Clinical features differ to :
a. 0-2 yearsBefore 2 years, the head enlarges excessively because the cranial sutures are
open. This enlargement is almost invariably the presenting sign.
1) Shape of Head: An abnormal head shape may suggest the diagnosis.Occipital prominence is seen in Dandy walker malformation. A
disproportionately large forehead is common with aqueductal stenosis. The
enlarged cranial size is quite evident and helpful in diagnosis.
2) Anterior Fontanelle:Normally the anterior fontanelle is small, depressed ina relaxed sitting patient but with hydrocephalus, it is enlarged and full even
when the infant is quiet and upright.
3) Sutures:palpation of spreading sutures also help in the diagnosis.4) Percussion Note: The percussion note of a normal infant skull is of a "Crack
pot", while with hydrocephalus it starts resembling a watermelon. This sound
is particularly striking when examiner places his ear against the infant's skull
while percussing.
5) Scalp Veins: The scalp veins are usually prominent, particularly in cryinginfants. The prominence is caused by compression of basal venous outlets by
increased pressure, which results in shunting of blood through the valveless
collateral system into easily distended scalp veins.
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6) The Eyes: As the hydrocephalus progresses, the eyes are displaced downwardby pressure on the thinned orbital roofs. This displacement of eyes causes the
sclera to be visible above the iris, termed as the "setting sun" sign.
7) Cranial Nerves: Optic atrophy is a common finding in advancedhydrocephalus due to the compression of the optic chiasma and the optic
nerve by dilated anterior third ventricle and increased intracranial pressure
(ICP). Abducent nerve paresis secondary to stretching is common. Nystagmus
and random eye movement may be present. Vision can also be affected by
damage to occipital cortex by grossly dilated occipital horns.
8) Muscle Tone and Deep Tendon Reflexes:With progressive hydrocephalus,the deep tendon reflexes and muscle
9) Growth Retardation: In hydrocephalic children, growth failure and delayedneurological development are common. Head and trunk control is particularly
affected.
b. More than 2 to 12 years
These patients fall into two groups differentiated by the presenting clinical
features:
1) The first group includes those children who have preexisting (infantile)unrecognized, progressive hydrocephalus with normal or retarded
neurological development. They are usually diagnosed after an incidental
head injury leading to rapid deterioration of neurological functions. They
usually have slightly enlarged heads with optic atrophy or papilloedema,
abnormal hypothalamic functions (short stature or gigantism, obesity, delayed
puberty, primary amenorrhea or menstrual irregularity and diabetes inspidus)
and spastic lower limbs. Performance IQ is worse than verbal IQ and learning
problems are common.
2) The second group consists of children who develop hydrocephalus afterclosure of cranial sutures; therefore, head circumference is usually within
normal limits. Papilloedema is a common finding due to raised ICP. Abducent
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nerve paresis and spasticity of lower extremities with hyper reflexia are also
noted. Morning headaches and vomiting are common.
Figure 2.4. Differential Diagnosis of Macrocephaly9
2.7.Diagnostic Procedures3,8,9Diagnostic procedures include :
1) Head Circumference: The head size should be measured by taking maximalobtainable circumference with measuring tape. The circumference is plotted
on a growth chart having head circumference column. This procedure is of
greatest importance in demonstrating an excessive rate of growth by serial
measurements
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2) Fusion of Sutures: Progressive hydrocephalus beginning before closure ofcranial sutures, prevents the fusion of sutures leading to continuous excessive
head enlargement.
3) Plain X rays: This modality is of great diagnostic value and will confirmmany clinical findings, such as an enlarged head, crainofacial disproportion,
wide spread sutures and large anterior fontanelle. Small posterior fossa with
low position lumboid sutures in aqueductal stenosis or large posterior fossa in
Dandy walker malformation may be found. In older children elongated
interdigitations of suture line indicates increased ICP. There may be evidence
of intracranial convolutional markings (silver beaten appearance) and
demineralization of dorsum sella.
4) Ventriculography: It is an invasive procedure in which the dye is injectedinto the ventricular system through lumbar puncture in order to see the size of
ventricles and flow of dye on plain x-rays. It has been replaced by ultrasound
and CT scan.
5) Pneumoencephalography: It is also an invasive technique similar toventriculography, only difference is that here air is injected into the cerebral
ventricles. This has also been replaced by non-invasive procedures.
6) Ultrasonography: It is a non-invasive procedure used only in patients inwhom the anterior fontanelle is open.
Figure 2.5. Ultrasonoghraphy in hydrocephalus9
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Figure 2.6. Normal Ultrasound9
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Figure 2.7. Abnormal Ultrasound9
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7) Computed Tomography: CT scan superceded other invasive investigationslike Ventriculography and Pneumoencephalography. It has a major role in
accurate assessment of ventricular size, extracerebral spaces and site of
obstruction.
Figure 2.8. CTScan in Hydrocephalus patient9
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8) Magnetic Resonance Imaging (MRI): This is also a noninvasiveinvestigation. It may also be used in antenatal diagnosis of Hydrocephalus.
Figure 2.9. Noncommunicating
obstructive hydrocephalus caused byobstruction of the foramina of Luschka
and Magendie. This MRI sagittal
image demonstrates dilatation oflateral ventricles with stretching of
corpus callosum and dilatation of the
fourth ventricle1
Figure 2.10. Noncommunicating
obstructive hydrocephalus caused by
obstruction of foramina of Luschkaand Magendie. This MRI axial image
demonstrates dilatation of the lateral
ventricles1
Figure 2.11. Communicating
hydrocephalus with surrounding
"atrophy" and increased periventricularand deep white matter signal on fluid-attenuated inversion recovery (FLAIR)
sequences. Note that apical cuts (lower
row) do not show enlargement of thesulci, as is expected in generalized
atrophy. Pathological evaluation of
this brain demonstrated hydrocephaluswith no microvascular pathology
corresponding with the signal
abnormality (which likely reflectstransependymal exudate) and normal
brain weight (indicating that the sulci
enlargement was due to increased
subarachnoid cerebrospinal fluid[CSF] conveying a pseudoatrophic
brain pattern)1
.
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Figure 2. 12. Noncommunicating
obstructive hydrocephalus caused by
obstruction of foramina of Luschka
and Magendie. This MRI axial image
demonstrates fourth ventricle
dilatation.1
Figure 2.13. 11 years old girls MRI who presented with three days of headache and
nousea/vomiting9
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several alternatives for repeated ventricular punctures but this procedure failed
due to a number of complications.
b. Open Ventricular Drainage: Wernicke in 18th century tried externalventricular drainage but due to high infection rate it was also abandoned.
c. Closed Ventricular Drainage: In order to avoid infection, many methods ofinternal drainage were devised. The modern surgical treatment had its origin
in last decade of 19th and 1st decade of 20th century. Most of the procedures
now in use such as peritoneal and circulatory shunting of CSF were conceived
and tried at that time. Excellent reviews of surgical treatment of
hydrocephalus have been published in journals by Dandy and Blackfan
(1914)21, Davidoff (1929)20, Jackson (1951)22, Scarff (1963)23 and
Balasubramanian (1967).24 The sites for diversion of CSF from the cerebral
ventricles is presented in Table 2.2.
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Table 2.2. The sites for diversion of CSF from the cerebral ventricles
Operation that bypass obstruction in the ventricular system include Third
Ventriculostomy and Cannulation of the aqueduct
Third ventriculostomy
This procedure is reserved for obstructive cases in patients who have normal
or near normal spinal fluid absorptive capacity. A blunt instrument is used to
penetrate the floor of the third ventricle. Sharp instruments or lasers can cause
vascular injury. Leaving a clamped drain in place postoperatively might be prudent.
The burr hole placed on the coronal suture allows a straight trajectory to the foramen
of Monro. Stereotactic guidance is not needed if endoscopic techniques are used.
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Indications for Shunt Surgery
The indication of surgical intervention are related to intracranial hypertension,
neurological dysfunction, evidence and degree of ventricular dilatation, the presence
or absence of pathological lesion, the nature and the location of obstruction. Some
surgeons insert the shunt in any patient with large ventricles while others restrict it to
those with potentially reversible deficit or progressive deterioration. Progressive
ventriculomegally on CT scan combined with observation of developmental deficits
in infants or intellectual and motor disability in older children are a few criteria.
Ventriculo peritoneal shunting
This is by far the most common procedure for cerebrospinal fluid diversion.
The abdomen should be able to absorb fluid. The ventricular catheter can be placed
more reliably from the coronal approach. Some surgeons still prefer parieto-occipital
catheters. The proximal catheter tip should lie anterior to the choroid plexus in the
frontal horn of the lateral ventricle.
Ventriculo-atrial shuntingThis procedure usually is the first choice for patients who are unable to have
abdominal distal catheters (e.g. multiple operations, recent abdominal sepsis, and
known malabsorptive peritoneal cavity). The procedure carries more risk and long-
term complications are more serious (eg, renal failure, great vein thrombosis).
Fluoroscopic guidance is necessary to prevent catheter thrombosis (short distal
catheter) or cardiac arrhythmias (long distal catheter).
Postoperative care
Postoperatively, patient is kept in horizontal position for 12-24 hours and then
gradually elevation of head is allowed depending upon condition of anterior
fontanelle. Oral intake is initiated within 12 hours if there is no abdominal distension
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and peristalsis is audible. Follow up includes regular fortnightly visits for first three
months and then after every month up to age of one year. From age of 1-3 years, 6
monthly visits and after that annual visits are required.
Figure 2. 14. Diagrom of VP and VA shunt7
Figure 2.15. Plain Films used to ais in confirming proper location of shunt in 3
years old boy s/p VP shunt placement9
2.9.Post-operative complications3,5,7The complications depend on the type of shunt and the underlying
pathophysiology. Infection is the most feared complication in the young age group.
The overwhelming majority of infections occur within 6 months of the original
procedure. Treatment of infected shunts with antibiotics alone is not recommended
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because bacteria can be suppressed for extended periods and can resurface once
antibiotics are stopped.
Subdural haematomas occur almost exclusively in adults and children with
completed head growth. Incidence of subdural hematomas can be reduced by slow
postoperative mobilization. Shunt failure is mostly due to sub optimal proximal
catheter placement. Occasionally, distal catheters fail. Headaches are caused by over
draining. Peritoneal cyst (Excess fluid collection in peritoneal cavity) may occur
because of poor absorption.
Figure 2.16. Symptoms of shunt problem7
2.10.Prognosis1,3,10Hydrocephalus is usually a lifelong disorder. Prognosis depends on a number
of factors, including the underlying condition, its duration and degree, as well as
response to treatment. The mortality rate in shunt-treated pediatric patients with
hydrocephalus remains high, dependent on the underlying cause for shunt insertionand the subsequent development of infection and other complications
In untreated hydrocephalus, death may occur by tonsillar herniation secondary
to raised ICP with compression of the brain stem and subsequent respiratory arrest.
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Shunt dependence occurs in 75% of all cases of treated hydrocephalus and in 50% of
children with communicating hydrocephalus. Patients are hospitalized for scheduled
shunt revisions or for treatment of shunt complications or shunt failure. Poor
development of cognitive function in infants and children, or loss of cognitive
function in adults, can complicate untreated hydrocephalus. It may persist after
treatment. Visual loss can complicate untreated hydrocephalus and may persist after
treatment.
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CHAPTER 4
DISCUSSION AND SUMMARY
`Hydrocephalus is characterized by an increased volume of CSF in association
with progressive ventricular dilation. In communicating hydrocephalus, CSF
circulates through the ventricular system and into the subarachnoid space without
obstruction. In noncommunicating hydrocephalus, an obstruction blocks the flow of
CSF within the ventricular system or blocks the egress of CSF from the ventricular
system into the subarachnoid space. A wide variety of disorders, such as hemorrhage,
infection, tumors, and congenital malformations, may play an etiologic role in the
development of hydrocephalus.
Clinical features of hydrocephalus include macrocephaly, an excessive rate of
head growth, irritability, vomiting, loss of appetite, impaired upgaze, impaired
extraocular movements, hypertonia of the lower extremities, and generalized
hyperreflexia. Without treatment, optic atrophy may occur. In infants, papilledema
may not be present, whereas older children with closed cranial sutures can eventually
develop swelling of the optic disk. Hydrocephalus can be diagnosed on the basis of
the clinical course, findings on physical examination, and CT or MRI scan.
Treatment of hydrocephalus is directed at providing an alternative outlet for CSF
from the intracranial compartment. The most common method is ventriculoperitoneal
shunting. Other treatment should be directed, if possible, at the underlying cause of
the hydrocephalus.
According to our case report, B, male, 10 days old came with enlarged head from his
birth, meconium (+) in the first 24 hours, history of vitamin K injection (-) history of
seizure (-) fever (-) vomiting (-) diarhea (-)Urination (+) N Defecation (+) black stool.
Patient was reffered and diagnosed with hydrocephalus. History of Pregnancy,
patient was third cild, mothers age when pregnant 34 years old, history of using
herbs (-) drugs (-) when pregnancy. History of labor, patient born aterm, section
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caesarian labor in hospital, spontaneous crying, blue baby (-) history of premature
rupture of membran not clear, birth weight 3300 gram, birth length 47 cm.
From Physical Examination found head circumference was 42 cm (> +2 SD)
and sunset ye phenomenom (+). Patient were diagnosed with hydrocephalus.
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7. Kings Collage Hospital. 2011. Hydrocephalus in children: its treatment usingthird ventriculostomy and ventricular peritoneal (VP) shunts : Information for
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9. Edwards, J., Lieberman, G., 2002. Hydrocephalus in Children : DiagnosticImaging and Radiological Characteristics. Harvard Medical School.
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10. Vinchon, M., Rekate, H., and Kulkarni3, A.V., 2012. Pediatric hydrocephalusoutcomes: a review. In : Vinchon et al. Fluids and Barriers of the CNS, 9:18
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