Volume 03 Issue 02-2023
32
International Journal of Medical Sciences And Clinical Research
(ISSN
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2771-2265)
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ABSTRACT
This article deals with the clinic, diagnosis and methods of treatment of occlusive hydrocephalus in children. The
authors of the article consider that surgical intervention in the treatment of occlusive hydrocephalus in children is the
only method to combat the disease. In most cases, medication can only slow down the course of the disease, but does
not eliminate the underlying cause of the disease. In the case of a successful operation, almost complete recovery and
a return to a normal life is possible.
KEYWORDS
Hydrocephalus, head, brain, treatment, surgery, neurosurgery, neurological and mental disorders, hematoma,
intraventricular tumour.
INTRODUCTION
Occlusive hydrocephalus is one of the most common
neurosurgical diseases of childhood [6; 7; 11].
According to different authors, the incidence of this
disease is 3-4 cases per 1000 newborns [5; 10]. In most
cases, hypertension-hydrocephalus syndrome is the
main cause of patient decompensation, leading to
Research Article
CLINIC, DIAGNOSIS AND TREATMENT OF OCCLUSIVE HYDROCEPHALUS
IN CHILDREN
Submission Date:
February 18, 2023,
Accepted Date:
February 23, 2023,
Published Date:
February 28, 2023
Crossref doi:
https://doi.org/10.37547/ijmscr/Volume03Issue02-07
Saidumarov Dilshod Mirzaakhmatovich
Doctor Neurosurgeon, Fergana Branch Of Republican Scientific Center Of Emergency Medical Care, Fergana,
Uzbekistan
Saidumarova Marguba Tulanovna
Assistant, Department Of Pathological Physiology, Fergana Medical Institute Of Public Health, Fergana,
Uzbekistan
Journal
Website:
https://theusajournals.
com/index.php/ijmscr
Copyright:
Original
content from this work
may be used under the
terms of the creative
commons
attributes
4.0 licence.
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severe, sometimes irreversible neurological and
mental disorders, which lead to persistent disability of
patients [3; 8], therefore, the search for effective
treatments for this disease is an urgent medical and
social task.
METHODS
Hydrocephalus was rarely described in ancient medical
literature, although its existence and symptoms were
well known. Hippocrates, the father of medicine, is
considered to be the first physician to attempt to
document the treatment of hydrocephalus. He
recommended trepanation for the treatment of
epilepsy, blindness and possibly hydrocephalus. The
Greeks reportedly treated hydrocephalus by wrapping
the bark around the patient's head and inserting it into
the trepanation orifices.
Evacuation of superficial intracranial fluid in children
with hydrocephalus was first described in detail by Ibn
Sina. Due to a poor understanding of the
pathophysiology of hydrocephalus, initial attempts at
therapy were sporadic and usually resulted in failure.
Many practitioners relied on conservative therapy.
Treatment attempts included many medications,
laxatives such as rhubarb, calomel and oil as well as
various diuretics, head wraps, bloodletting and cranial
trepanation [2].
In neurology, the term "hydrocephalus" currently
refers to the increased accumulation of cerebrospinal
fluid (cerebrospinal fluid) in the cranial cavity. If the
cause is an occlusion (blockage, compression) of the
cerebrospinal fluid pathways, hydrocephalus is called
occlusive hydrocephalus. A synonym for this condition
is closed hydrocephalus, as occlusion leads to the
occlusion of the cerebrospinal fluid system and the
accumulation of cerebrospinal fluid. In the ICD-10,
occlusive hydrocephalus is listed under the name
'obstructive'. The disorder is secondary and always has
a causal pathology. Occlusive hydrocephalus occurs at
all ages, in children and adults, and may be congenital.
In some cases there is an acute occlusion that requires
immediate medical attention.
The cerebrospinal fluid system consists of four
ventricles: the paired lateral ventricles and the
unpaired third and fourth ventricles. Cerebrospinal
fluid from the lateral ventricle enters ventricle III
through the interventricular foramen of Monro, then
through the aqueduct of Sylvius into ventricle IV, from
which it drains through the Luschka and Magendie
orifices into the cerebral and spinal subautery cisterns.
Occlusive hydrocephalus develops when there is an
obstruction in any part of the described cerebrospinal
circulation system. Factors of occlusion may include:
•
Abnormal
development
of
the
brain.
Congenital stenosis, underdevelopment of the
aqueduct of Sylvius, Dandy-Walker syndrome, and
Arnold-Chiari anomaly are genetically determined or
formed under conditions of intrauterine infection, fetal
hypoxia, and teratogenic influences. Anatomical
changes in these malformations lead to the
development of hydrocephalus in the antenatal period
or shortly after birth.
•
Cerebral tumors. Ventricular neoplasms reduce
ventricular volume and cause occlusion of the
communicating orifices. Cerebral cistern tumours
prevent the flow of cerebrospinal fluid from the
ventricles into the cisterns. Pericentricular tumors,
neoplasms of the trunk, cerebellum squeeze the
cerebrospinal fluid pathways as they grow. The result
of these processes is an accumulation of cerebrospinal
fluid in the ventricles.
•
Intracerebral hematoma. Formed due to
craniocerebral trauma (including intracranial birth
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trauma),
haemorrhagic
stroke.
Occlusive
hydrocephalus is caused by the occlusion of the
cerebrospinal fluid outflow pathways by the
hematoma that has formed.
•
III ventricular colloidal cyst is a benign
neotumoral entity. The cyst increases in size and blocks
the entrance to the aqueduct of Sylvius. The outflow
tract is blocked and hydrocephalus develops.
•
Hemorrhage in the ventricles of the brain.
Occurs with trauma, rupture of the vessels of the
arteriovenous malformation, hematoma bursting into
the ventricles. The blood in the ventricular cavity
coagulates to form clots that occlude the Monro,
Luschka, Magendie orifices and the narrow canal of the
cerebral aqueduct.
Obstruction, compression of the liquor-bearing tracts
leads to a disturbance of the outflow and accumulation
of cerebrospinal fluid in the ventricles of the brain. The
ventricular volume increases, and with the intracranial
space closed, this leads to an increase in pressure
inside the cranial cavity. The rate of increase in
intracranial hypertension depends on the extent and
mechanism of the occlusion. Monroe orifice occlusion
leads to lateral ventricular enlargement, occlusion at
the level of the aqueduct of Sylvius leads to dilation of
the III and both lateral ventricles, and at the level of the
Magendie and Luschka orifices leads to total dilatation
of the ventricular system.
Occlusive hydrocephalus of tumour origin forms
gradually, with post-traumatic haematoma within a
few days. Occlusion by a blood clot, part of an
intraventricular tumour occurs suddenly, leading to
acute hydrocephalus. Intraventricular neoplasms,
colloidal cysts can cause occlusive crises, a transient
blockage of the cerebrocirculation that occurs when
the mass is displaced. Significant intracranial
hypertension causes compression of brain tissue,
feeding vessels. Hypoxia and dysmetabolic changes
occur, leading to neuronal death. The continuing
increase in pressure causes displacement of cerebral
structures
(mass
effect),
leading
to
severe
complications.
In clinical practice, occlusive hydrocephalus is divided
into
etiological
and
anatomotopographic
classifications. Both classifications have implications
for the choice of the most appropriate treatment
modality. According to the etiological principle, a
distinction is made:
•
the congenital form - formed during the
intrauterine period due to malformations of the spinal
cord system, cerebral anomalies (e.g. brain cysts) that
squeeze the liquor-bearing tracts. Appears from the
first days of life.
•
acquired form - occurs during life, caused by
trauma and CNS diseases. Appears in conjunction with
other symptoms of the causal pathology.
According to anatomotopographic classification,
occlusive hydrocephalus is subdivided into:
•
monoventricular - one lateral ventricle is
dilated. The cause is a blockage of the interventricular
orifice.
•
biventricular - the volume of the two lateral
ventricles is enlarged. Occlusion at the level of the third
ventricle.
•
triventricular - enlargement encompasses
three ventricles. Obstruction of the liquor flow is
located in the area of the cerebral aqueduct.
Volume 03 Issue 02-2023
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•
tetraventricular - all ventricles are dilated. The
cerebrospinal circulation is impaired at the level of the
outflow pathways from the IV ventricle.
Regardless of the level of cerebrospinal block, the
clinical picture is dominated by signs of intracranial
hypertension. Patients present with intense headache
(cephalgia), non-meal related nausea, vomiting, and
frequent nosebleeds. Cephalgia causes a forced
position of the head and is accompanied by a feeling of
pressure on the eyeballs. The onset of symptoms is
acute or subacute, with the background of the clinical
picture of the underlying pathology. In some cases,
occlusive hydrocephalus is the first sign of malignancy.
The cochleovestibular and optic nerves are most
commonly affected by intracranial hypertension.
Vestibular ataxia, tinnitus, hearing loss, visual acuity
and visual field defects are observed. Epileptic
paroxysms are not uncommon. Associated focal
deficits depend on the causal pathology and are
represented
by
paresis,
paralysis,
sensory
disturbances, cognitive deficits and cerebellar
syndrome. The cerebral block at the level of the third
ventricle is characterized by diencephalic symptoms:
pulse palpitations, blood pressure fluctuations,
hyperhidrosis, discoloration of the skin (pallor,
hyperemia). Obturation of the Sylvian aqueduct is
accompanied by dissociation of the pupillary response
to light, impaired convergence and gaze paresis. Block
in the fourth ventricle is accompanied by cerebellar
ataxia.
Occlusive hydrocephalus in young children is
manifested by an increase in the size of the skull,
divergence of the cranial sutures, enlarged and swollen
fontanelles.
Typical
features
of
congenital
hydrocephalus are an enlarged globe-shaped head, a
relatively small torso, deep eye sockets and swollen
scalp veins. The children are retarded in their
psychophysical
development.
The
severity
of
intellectual disability depends on the age of onset,
duration, severity of intracranial hypertension.
Occlusive hydrocephalus can be accompanied by an
acute and almost complete block of cerebrospinal fluid
flow - occlusive hydrocephalus crisis. The attack is
accompanied by acute intense cephalgia, repeated
vomiting, facial hyperaemia followed by pallor,
oculomotor disturbances, depression of consciousness
and autonomic symptoms. The most severe
complication of hydrocephalus is the mass effect.
Displacement of brain tissue in the direction of the
greater occipital foramen leads to compression of the
medulla oblongata, which are located vital centres
regulating cardiovascular and respiratory activity. A
violation of the functions of the latter is fatal.
Diagnostic activities begin with the collection of
anamnesis: determining the time of the onset of the
intracranial pressure increase symptoms, the nature of
their development, the presence of a diagnosis of brain
disease, the fact of head trauma, etc. A further
diagnostic algorithm includes:
•
neurological examination. It allows the
neurologist to identify objective symptoms of
intracranial hypertension, existing focal deficits. The
findings allow a topical diagnosis to be made.
•
consultation with an ophthalmologist. It
includes ophthalmoscopy, perimetry, visometry.
Examination of the ocular fundus determines
congested optic discs, with prolonged hydrocephalus -
signs of optic atrophy. Examination of the visual fields
reveals narrowing, loss of certain areas, and visometry
reveals a decrease in visual acuity.
•
echoencephalography. Due to its ease of
execution it can serve as a screening method. Allows
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diagnosis of increased intracranial pressure, ventricular
dilatation, and displacement of cerebral tissues.
•
neuroimaging. Infants are examined by
neurosonography through the fontanelle, while others
are examined by MRI of the brain. The examination
makes it possible to diagnose malformations, localize a
cerebral block, and determine its cause. MSCT and CT
scan of the brain are performed in complex diagnostic
cases in addition to MRI, if there are contraindications
to MRI examinations.
It is necessary to differentiate occlusive hydrocephalus
from subarachnoid haemorrhage and other forms of
hydrocephalus. Differential diagnosis is also made
among the possible causes of occlusion. In children in
the first months of life, hydrocephalus must be
differentiated from macrocranial, which is mostly
familial, without symptoms of hypertension or
developmental delay.
The only effective treatment is neurosurgery
Since the 1950s, the standard treatment for any form
of hydrocephalus has been bypass surgery to restore
the movement of the cerebrospinal fluid. After cranial
trepanation, one end of the shunt, ending with a
radiopaque catheter, is inserted into the dilated
ventricular cavity. The intermediate, longest part,
made of silicone, is placed subcutaneously. The distal
end, which also has a catheter, opens into the
abdominal or thoracic cavity to allow drainage. The
shunt is fitted with a pump that automatically
regulates the pressure of the cerebrospinal fluid [1; 9].
Since the mid-1980s, endoscopic surgery has played a
significant role in the treatment of hydrocephalus.
The treatment of occlusive hydrocephalus by shunt is
quite effective, however, according to various sources,
complications during this operation amount to 40-60%
of cases. Depending on the cause of the dysfunction,
all or parts of the shunt must be replaced. Experience
shows that the most frequent complications that
require shunt revision occur between six months and
one year after surgery. Most patients who undergo
bypass surgery have to undergo several surgical
interventions during their lifetime. In any case, at least
two or more revisions should be expected - after all,
the child is growing. After bypass surgery, the patient
becomes shunt dependent, meaning that the rest of
his or her life will depend on the operation of the shunt.
Complications of bypass surgery:
•
occlusion (blockage) in both the ventricles of
the brain and the abdomen;
•
infection of the shunt, ventricles, cerebral
membranes;
•
mechanical damage to the shunt;
•
hyperdrainage
(rapid
discharge
of
cerebrospinal fluid from the ventricles) is often
accompanied by rupture of the convexital
veins and formation of hematomas;
•
hypodrainage (slow ventricular outflow tract) -
surgery is then ineffective;
•
development
of
epileptic
syndrome,
abdominal decubitus, etc.
Endoscopic treatment of hydrocephalus
At present, endoscopic treatment of hydrocephalus is
a priority in the world practice of neurosurgery. It is
worth mentioning the effectiveness of endoscopic
ventriculocysternostomy of the floor of the third
ventricle in treating occlusive hydrocephalus. This
operation is widely used and accounts for about 80% of
neuroendoscopic surgeries. The aim of the operation is
to create outflow pathways from the ventricular
system of the brain (ventricle III) into the cisterns of
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the brain, through which fluid is reabsorbed
(absorbed) as in a healthy person.
Indications for surgery:
•
primary operation in occlusive hydrocephalus
with the level of occlusion from the posterior
third ventricle and beyond;
•
alternative surgery for complications of bypass
surgery with removal of the previously
installed bypass system (instead of "bypass
revision" surgery);
•
post-traumatic hydrocephalus;
•
mixed hydrocephalus (internal and external);
•
operation of choice when removing the shunt
system to achieve shunt-independence;
Advantages of the operation compared to classic
shunts:
•
the operation restores the physiological (as in
a healthy person) liquor flow from the
ventricular system of the brain to the basal
cisterns;
•
no foreign div (shunt system) is implanted
into the div, thus avoiding related problems
(infection, malfunction, need for revisions);
•
a much lower risk of hyperdrainage and related
complications
(subdural
hematomas,
hydromas, etc.);
•
the operation is less traumatic;
•
the operation is more cost-effective for the
hospitals;
•
improved quality of life.
In addition to the above-mentioned treatments for
occlusive hydrocephalus, the correction of the
pathology of the liquor ducts (the plasticization of the
aqueduct of Sylvius lends itself to this method) as well
as the elimination of the blocking cause must also be
mentioned. If the volume of the mass is large, this type
of intervention is too traumatic. It can be performed in
case of hematomas, tumours. In an emergency,
external ventricular drainage of one of the lateral
ventricles is performed.
CONCLUSIONS
Surgical intervention is virtually the only method of
combating the disease. In the majority of cases,
medication is only able to slow down the progression
of the disease, but does not eliminate the underlying
cause. If the operation is successful, a nearly complete
recovery and a return to a normal life is possible.
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