Hidrocephaly
Hidrocephaly
Hidrocephaly
Mohan Dumitru
Hydrocephaly/Hydrocephalus
History
The term “hydrocephaly” comes from the Greek words: “hydro”, meaning “water” and
“cephalus”, meaning encephalon. As it results from the name, hydrocephaly is a pathological
state whose main characteristic is excessive accumulation of liquid at the level of the
encephalon/brain.
The cerebrospinal fluid (CSF) lies in the depths of the encephalon/brain and of the spinal
marrow, filling in the cavities called cerebral ventricles and the spinal canal.
There are four ventricles at the level of the encephalon: two lateral or teleencephalic, the
third ventricle or diencephalic and the fourth ventricle or rhombencephalic.
These ventricles interrelate with each other, with the spinal canal and the subarachnoidian
space surrounding the central nervous system.
Ventricle III
The third cerebral ventricle (III), situated on the midline, interhemispherically, is a tall slit
saggitally oriented, separating the anatomic formations of the diencephalon. It communicates
in the front side through the interventricular foramina of Monro, measuring 4-5 mm in length
and 3-4 mm in diameter, with the lateral ventricles and in the back side with the aqueduct of
Sylvius.
The Sylvius Aqueduct, the connection between ventricle III and ventricle IV, long of
approx. 2 mm and approx. 1,5 mm in diameter, represent one of the “sensitive” areas of the
ventricular system.
Ventricle IV
Ventricle IV is a rhomboid cavity, displaced between the dorsal faces of the bulb and
bridge, ventrally and the cerebellum dorsally. Enveloped by the ependimus it continues
caudally with the medullar/spinal canal. Ventricle IV communicates rostrally with the cerebral
aqueduct of Sylvius. This ventricle has a great importance in the cerebrospinal fluid
1
circulation. At the level of the fourth ventricle the link between the endoneural and the
subarachnoid sectors of the liquidian spaces is realized, through three foramina:
2
The Magendie foramen, punched in the fourth ventricle’s roof. Situated on the midline, it
realizes a large communication between ventricle IV and the cisterna magna, as the main
communication way between the ventricular system and the subarachnoidian space.
The Luschka foramina situated at the distal end of the fourth ventricle’s lateral recesses,
performs the connection between the fourth ventricle and the pontomedullary cistern. The
Luschka foramina are partially occupied by the choroid plexuses extensions, which thus
slip into the subarachnoidian space.
The 35-38 mm long fourth ventricle has two walls, four margins and four angles.
The Arachnoid Villi or Granulations are some digitiform evaginations of the arachnoid that
open up after crossing the durra mater in the sanguine lacunas of the diploia. As a general
rule they are displaced in systems of parallel tubes, juxtaposed and interconnected. Invisible
with new-born and children, they increase in dimensions progressively, exceeding the limit of
discrete optical detection, towards the age of 7-10 years.
These formations, more visible with ageing, would hyalinise, fibrosise and impregnate
with calcium insoluble salts, called Pacchioni arachnoidian granulations.
These corpuscles, with an incontestable role in the drainage of the cerebrospinal fluid to
the venous system, are placed in large numbers on both sides of the superior longitudinal
sinus which they invade. More rarely, they appear next to the lateral, cavernous and superior
rocky sinuses and in the confluence zone of the sinuses.
Definition
Hydrocephaly is defined as an abnormal accumulation of cerebrospinal liquid/fluid
(CSF/L), endocranial and in the specially constituted anatomical spaces, caused by a certain
circulation disorder, resorption or hyperproduction of the same, having as consequence the
appearance of a more or less specific symptomatology.
The incidence of this disease in the general population is of 1-1,5% and for the
congenital hydrocephalies it is of 0,2-3,5/1000 births.
Classification of hydrocephaly
According to the functional criterion, hydrocephaly is classified in two types:
- obstructive
- communicating
Obstructive hydrocephaly defines any action restricting the flux inside or from the
ventricular system. So a blockage anywhere along the ventricular paths (the interventricular
Monro foramen, the Sylvius aqueduct, the Magendie foramina from the fourth ventricle),
produces obstructive hydrocephaly with the enlargement of the ventricles located proximal to
the obstruction.
Any interruption of the liquidian flux after the CSF’s exit from the ventricular system is
called communicating hydrocephaly. It occurs in the case of cisterns obstruction, along the
subarachnoidian space or at the level of the arachnoid villosities.
The old classification grouping hydrocephalies into congenital and acquired is still valid today.
At present, the congenital hydrocephaly may be currently diagnosed by the intrauterine
echography of the fetus. The intracranial pressure is determined by the volume of the
cerebral tissue, the CSF volume, the circulant blood volume as well as by the other
intracranial tissues’ volume. An increase in volume for any of the components leads initially to
the compensating drop of the other’s volume, finally producing a constant growth of the
intracranial pressure.
The braincase content may be divided into three compartments:
- cerebral parenchyma
- vascular tree
- liquidian sector
The vascular system is the only system open to the exterior through the carotid arteries, the
vertebral arteries and the jugular veins. It may be compressed, thus, rapidly changing
volume.
The cerebral parenchyma may also alter its volume, but in the longer term, either through
cellular multiplying during brain development, or by cellular destruction, irrespective of the
mechanism causing it.
The liquidian system is composed of CSF and extracellular liquid. They are interdependent
on either side of the ependimus/ependyma wall and of the perivascular spaces. There are
times when the liquid volume grows abnormally, as it is in the cerebral atrophies and focal
porencephalies; in these cases CSF passively fills up the void created by the destruction of
the cerebral parenchyma. These situations are not consequences of a CSF dynamic
disturbance and do not embody pathological entities that are part of hydrocephaly.
Physiopathology
Hydrocephaly is the result of three mechanisms:
- hyperproduction of CSF
- producing resistance to CSF flowing
- occurrence of a CSF absorption deficit by increase of the venous pressure
The consequence of these mechanisms is increase of CSF pressure in order to maintain the
balance between the secretion and resorption debits. Ventricular dilatation is not the result of
unevenness between secretion and resorption but is secondary to the CSF hydrostatic
pressure growth.
The mechanisms producing dilatation of the ventricular system upstream to the obstacle
placed in the way of the CSF circulation path are several and they occur at different times of
disease progression. Ventricular dilatation is the result of the vascular system compression or
of a modified distribution of CSF and of the extracellular liquid inside the skull.
During a longer period, under the effect of compression, the cerebral parenchyma suffers
cerebral destructions and participates in the ventricles dilatation. With children, the exercise
of an abnormal pressure on the still unossified cranium sutures leads, secondarily, to an
increase of the cranial volume.
Etiopathogeny
Child hydrocephaly has two main causes:
- prenatal
- postnatal
The prenatal causes are responsible for the congenital hydrocephaly but also for the one
appeared postnatal or only belatedly manifested at adolescence. Etiology is usually
malformative, infectious or vascular and sometimes idiopathic.
Malformative causes:
- stenosis of the Sylvius aqueduct
- malformation Chiari type I and II
- malformation Dandy-Walker
- agenesis of the Monro foramina
The most common infectious causes are:
- purulent meningitis
- TBC meningitises
- parasitoses
Vascular causes determine hydrocephaly subsequent to:
- subarachnoidian hemorrhages or
- intraventricular hemorrhages of different etiologies
Postnatal causes for the development of hydrocephaly are the most frequent.
These are:
- tumoral expansive processes which in children represent the cause of over 20% of
hydrocephalies. The disease may be secondary to the evolution of the following tumor
types:
colloid cyst
choroid plexus papilloma
meduloblastoma
ependymoma
sellar region tumors with suprasellar extension
arachnoidian cysts
- the non-tumoral expansive processes are represented by:
vascular malformations
Galen vein aneurysms.
Adult hydrocephaly has a really varied etiology, congenital or acquired. The most frequent
causes are:
- meningeal hemorrhages
- meningitis
- cranial traumatisms
- neurosurgical interventions with opening of the ventricular system
- intracranial tumors (colloid cysts, craniopharyngiomas, hypophyseal adenomas,
epidermoid cysts, pineal region tumors, tumors of the posterior cerebral fossa, tumors
of the meninx developed on the tentorial incisure)
- intrarachidian tumors
Other rare causes with adults are:
- non-tumoral stenosis of the Sylvius aqueduct
- Dandy-Walker and Chiari malformations
- Sarcoidosis
- Paget’s disease (creates bony origin venous compressions), etc.
Paraclinical explorations
Invention and spreading of CT-scan and of the nuclear magnetic resonance imaging have
revolutionized the diagnosis and post-operational follow-up of hydrocephalies.
The CT-scan exam is the first step of the diagnosing process. Besides the fact that it reveals
a possible cause of the hydrocephaly, it allows to appreciate the ventricular dilatation and the
aspect of other subarachnoidian spaces.
The MRI exam completes the CT-scan allowing the precise description of a alleged
obstructive lesion. The typical exam is to highlight the Sylvius aqueduct stenosis impossible
to assess by a CT-scan. Besides the very detailed morphological aspects the MRI exam
allows a dynamic exploration of the CSF flux.
The medical treatment aims at stopping the evolution of hydrocephaly, reducing the CSF
production by administration of carbon anhidrasis inhibitors (acetazolamid) and diuretics
(furosemid), or by monitoring the resorption increase by administration of izosorbide-dinitrate.
The medical treatment indications are: infections of the shunts that require ablation,
monitoring the hydrocephaly until the next intervention of derivation; the medical treatment is
also useful in the symptomatic treatment of hydrocephaly subsequent to subarachnoidian
hemorrhages; to postpone the surgical intervention for a proper moment.
A timely treatment of an acute hydrocephaly with intracranial hypertension syndrome is
always successful, overlooking, of course, the cause of the disease. The hydrocephalies
treatment prognosis is directly dependant on the frequency and the severeness of the shunt’s
possible complications, meaning:
- mechanical complications (tubulature obstructions, tubulature detachment or
migration, etc.)
- infectious complications
- complications caused by excessive drainage of the CSF (subdural hematomas).