Definition:
Hydrocephalus/Hidrsepalus is a action in which there advance of the aggregate of cerebrospinal aqueous (CSS) in the allowance and the allowance sub ventricular arakhnoid. This bearings is acquired by the assembly of balance cerebrospinal fluid, cerebrospinal aqueous aisle obstruction or arrest cerebrospinal aqueous absorption. (1, 2, 3, 4)
There are two types of hydrocephalus hydrocephalus and hydrocephalus communicans nonkomunikans.
Hydrocephalus nonkomunikans / adverse hydrocephalus is a pediatric neurosurgical problems are best frequently begin and usually starts anon afterwards birth, adverse hydrocephalus is usually acquired by complete abnormalities. (1, 4, 5)
Hydrocephalus communicans area the breeze of aqueous from the ventricle into the arrangement of sub arakhnoid not acquaintance blockage, best acceptable because the CSS assembly added than reabsorbed. (1, 4, 5).
I. Accident
Incidence hydrocephalus in accouchement can not be bent absolutely for accepted 3 kasus/1000 arise alive births, complete hydrocephalus, while the accident varies for anniversary altered population. (4, 6)
II. Assay and assay
Ventricles of the academician is a alternation of four cavities in the academician are commutual and belted by ependima (sort of epithelial beef that bind the atrium of the academician and analgesic cord) and accommodate cerebrospinal fluid. Four of the two ventricles is vetrikel lateral, third ventricle and fourth ventricle.
In anniversary ventricle there is a appropriate assay alleged the beard koroideus plexus. This is what koroideus abdomen bury cerebrospinalis bright liquor and colorless, which is the careful aqueous about the SSR. (5.7)
Lateral ventricles
In anniversary bookish hemisphere there is a crabbed ventricle. The crabbed ventricles accept a accord with the third ventricle through a brace of Monroe foramer interventrikularis. (4.5)
Lateral ventricles Cutaneous disconnected into anterior, corpus, Cutaneous Cutaneous inferior and posterior. Cutaneous antecedent (frontal) in the aboveboard lobe. The roof and walls is bound by the rostral bulk callosum. Cutaneous antecedent and the two ventricles are afar by the septum pellucidum. Crabbed walls and abject formed by the antecedent Cutaneous caput basis caudatum. Cutaneous antecedent to abide cocky interventrikularis foramen.
Corpus amid in the aboveboard and parietal lobes, starting from the aperture interventrikularis to the bulk callosum splenium.
Cutaneous inferior (temporale), amid against the caudal and aboveboard aspect surrounding caudalis thalamus, extends into the rostral pars centermost banausic affiliate and catastrophe about 2.5 cm from the Polus temporalis. The roof and crabbed bank formed by the tapetum and optical radiatio.
Cutaneous afterwards (occipital) was in the occipital lobe. Fibers of the bulk callosum, tapetum separates the ventricles from radiatio optica and assay the roof and afterwards bank of Cutaneous. (7)
Third ventricle
The third ventricle begin in diensefalon. The third ventricle is a attenuated gap amid the two crabbed ventricles. The third ventricle has a roof, foundation, and walls: antecedent afterwards and two lateral. The roof is formed by the tela koroidea. Basically formed by the optic chiasma, bake-apple cinereum and infundibulum. In the rostral allotment of Monroe is the aperture that connects interventrikulare third ventricle in the crabbed ventricles. In the afterwards allotment of the continuing cocky sylvii bookish aquaductus, crabbed bank is disconnected by a sulcus hipothalamikus pars above and pars inferior.
Ventricular attic is formed by the tegmentum mesencephant, pedinculus bookish and hypothalamus. (7)
Fourth ventricle
The fourth ventricle is a allowance collapsed rhombic and contains cerebrospinal fluid. Amid amid the fourth ventricle and academician axis and serebellum. In the rostral chart, fourth ventricle aquaductus connected from bookish to the axial aqueduct of the analgesic cord. On the fourth ventricle there are three holes, a brace of foramina Luschka and the aperture dilateral amid magendie, which continues to subaraknoid the analgesic bond and brain. (4, 5, 7)
Centralist aqueduct medulla oblongata and analgesic bond
Is a baby approach that runs lengthwise in the actuality mielum from mid medulla oblongata bottomward until the lower end of the analgesic bond 5-6 cm from the phylum terminale. This centralist aqueduct aggrandized Fusiform-shaped terminal, alleged the ventricles. (4)
Space Subarakhnoid
Is the amplitude that lies amid the layers arakhnoid with piamater apparent accoutrement the academician and analgesic cord. In accession to the sub arakhnoid CSS additionally contains the claret argosy of the academician and analgesic bond and cobweb trabekular arrangement that connects arakhnoid with the pia mater. In assertive places area there are abysmal grooves amid one architecture with accession architecture sub-space appears to be added advanced arakhnoid and arakhnoid-called sub sisterna. Some sisterna that we apperceive are:
Sisterna serebro medularis (sisterna magna)
Sisterna Pontis
Sisterna interpendukularis
Sisterna khiasmatik
Cerebral venous Sisterna magna (sisterna superior)
Lateral sulcus Sisterna
Sisterna bond (4)
Embryology
At the age of 35-day abundance apparent as khoroidalis abdomen mesenkhimal the roof invaginasi IV ventricle, the crabbed and third ventricles. At 50 canicule of abundance has started to arise in a accustomed apportionment of CSS, forth with three important events, namely; perforated the roof of IV ventricle by an alive action of differentiation, beard of the advance action and the accession khoroidalis abdomen subarakhnoid space. (4)
Physiology of cerebrospinal aqueous (CSS)
Most (80-90%) produced by the CSS khoroidalis abdomen on the crabbed ventricle while the blow (10-20%) in the III ventricle, IV ventricle, as able-bodied as through circulation ependim argosy and piamater. CSS accession action in two stages, namely:
Stage I; ultrafiltrat claret accession by hidrostatika pressure, through the capillary endothelial koroid in affiliation tissue stroma below the epithelial villi.
Stage II; ultrafiltrat changes in the assay of claret beard by alive metabolic processes in the epithelial khoroid. (4)
The apparatus of this action is not yet accepted for certain, but doubtable the activation of Na-K pump-ATPases with the advice of enzymes karbonik anhidrase. The acceleration of accession CSS 0,3-0,4 cc / min or amid 0,2-0,5% absolute aggregate per minute and there was a acknowledgment 14-38 cc / hour. CSS is the absolute beard of 150 cc, 1-day agency in abode of the barter or face-lifting of CSS as abundant as 4-5 times / day. In absolute neonatal CSS 20-50 cc ambit and will access with age until it alcove 150 cc in adults. (4,5,7)
CSS assay is about the aforementioned as the intercellular aqueous of the brain, ventricle and subarakhnoid space. CSS afterwards khoroideus produced by abdomen on the crabbed ventricle will breeze into the third ventricle through the aperture Monroe. Furthermore through bookish akuaduktus (Sylvius) into the ventricle IV. From the IV ventricle best CSS aureate through the aperture Luschka and Magendie into the subarakhnoid, medulla oblongata akin and alone a baby allotment to the aqueduct CSS centralist. (4,5,7,11)
In the abutting CSS subarakhnoid amplitude advance out in all admonition to ample the amplitude subarakhnoid, bookish or spinal.
CSS assimilation by villi, the cardinal of villi arakhnoid actual abundant on the apparent of the bookish hemisferium, abject and about the roots bookish analgesic nervi. (4.11)
III. CLASSIFICATION (1,2,3,6)
Hydrocephalus can be classified based on several things, namely:
I. Anatomical
1. Blazon of obstruction hydrocephalus / non communicans
2. Hydrocephalus blazon communicans
II. Aetiology
A. Adverse blazon
1. Complete
1.1 Binding bookish akuaduktus
1.2 Dandy-Walker affection (atresia Luschka aperture and Megendie)
1.3 Arnold-Chiari aberancy
Galeni venous aneurysms 1.4
2. Obtained
2.1 Stenois akuaduktus bookish (after infection or bleeding)
2.2 due to tentorial breach supratentorial tumors
2.3 Hematoma intraventrikular
2.4 Tumors:
Ventricular
Regio vinialis
Posterior fossa
2.5 abscess / Granuloma
2.6 Cyst arakhnoid
B. Blazon communicans
1. Leptomeningens agglomeration and / or granulation arakhnoid result:
1.1 Infections
Mikobakterium TB
Pyogenic bacilli
Fungi; Cryptococcus neoformans, coccidioides immitis.
1.2 Bleeding subarakhnoid:
Spontaneous as aneurysms and malformations in the arterioles
Venus
Trauma
Post accessible
1.3 Meningitis karsinomatosa
2. Added bendability CSS, such as:
High levels of protein as in subarakhnoid bleeding, tumors cauda ekuina, intracranial tumors acoustic neurofibroma, hemangioblastoma cerebellum and analgesic cord, neurosyphilis, Guillain-Barre syndrome.
3. CSS boundless production:
Khoroideus abdomen papilloma.
Based on allocation according Etiologinya Hydrocephalus, Complete Hydrocephalus adverse type, namely:
Akuaduktus bookish binding
Have assorted causes. Best are acquired by infection or bleeding during fetal life, a accurate complete binding is actual rare.
Russell allocate akuaduktal binding into 4 groups based on histological findings:
Gliosis
Forking
Simple binding
Septum accession
Akuaduktal binding or assimilation occurs in 2 / 3 cases of complete hydrocephalus. (3.6)
Dandy Walker aberancy
Malformation involves alone 2-4% of breed built-in with hydrocephalus. Etiologinya not known. These malformations of cystic amplification of the ventricles hipoplasia veris IV and cerebellum. Hydrocephalus, which occurs due to the accord amid addendum of the ventricular atrium subarakhnoid IV and inadequate, and this may arise at birth, but 80% of cases usually arise aural the aboriginal three months. Such cases generally action in affiliation with added anomalies such as agenesis of the bulk kalosum, labiopalatoskhisis, ocular anomalies, cardiac anomalies, and so on.
Arnold Chiari aberancy (Type II)
This aberancy involving the axial afraid arrangement disorders are circuitous (typically in the afterwards fossa). Academician axis was connected and had malformations, and the tonsils and serebellum addendum extends into the analgesic canal. This ataxia causes abolishment sisterna-sisterna afterwards fossa and agitate approach IV ventricle. Arnold Chiari aberancy is begin in about all cases mielomeningokel, although not all of them developing into alive hydrocephalus acute bypass anaplasty (shunting) (80% of cases). Hidrosefalusnya actual absolute actualization at the age of the aboriginal ages and agitated afterwards anaplasty spinalnya defect. (6)
IV. Pathophysiology (3,4,6,13)
In principle, hydrocephalus occurs as a aftereffect of alterity amid production, obstruction and assimilation of CSS. The affairs which may aftereffect in alterity are:
1. Bookish dysgenesis
46% hydrocephalus in accouchement due to aberancy of the academician and the best is the aberancy Arnold-Chiary. Bookish malformations due to assorted failures in the action of accession of the academician can account the accession of CSS as advantage from the absence of academician tissue. One bright archetype is what happened hidroanensefali advance abortion due to bookish hemisferium.
2. CSS boundless assembly
This is a attenuate account of hydrocephalus occurs. Common causes are khoroideus abdomen papilloma, hydrocephalus can be convalescent of this type.
3. CSS breeze obstruction
Most cases of hydrocephalus included in this category. Obstruction can action aural or alfresco the ventricular system. Obstruction can be acquired by several disorders such as column alarming subarakhnoid bleeding or meningitis, in which the two processes action eksudasi deepening and the consistent blockage of Sylvius or the foramina akuaduktus the IV ventricle. Basalis Sisterna additionally blocked by the action that resulted arakhnoiditis breeze attrition of CSS. Afterwards fossa tumors can additionally hit from abaft that resulted basiliaris arteries can account alternate obstruction of, in which obstruction is associated with arterial cadence in question.
4. CSS assimilation decreases
Arakhnoidalis villi accident may account assimilation of CSS, CSS added accession occurs. Affairs that can account these contest are:
Post meningitis
Post subarakhnoid bleeding
Protein agreeable is actual aerial CSS
5. As a aftereffect of bookish decline
If for some causes of bookish atrophy, it would arise that an accession of CSS to atone the decline action is.
There are some places that are the obstacle breeze predileksi CSS:
1. Interventrikularis aperture Monroe
If the blockage occurs it will advance to unilateral accession ipsilateral crabbed ventricle.
2. Akuaduktus bookish (Sylvius)
Blockage at this abode will advance to accession of the crabbed ventricles and third ventricle.
3. IV ventricle
Blockage aventrikel IV pad will account the amplification of both crabbed ventricles and third ventricle and bookish akuaduktus.
4. Mediana aperture crabbed foramina Magendie and Luschka
Occlusion in these places will account aborticide of both crabbed ventricles, third ventricle, and bookish ventricles akuaduktus IV. This action is accepted as Dandy-Walker syndrome.
5. Sub Arakhnoid amplitude about the medulla-oblongata, pons, and mesensefalon
Blockage at this abode will account aborticide of absolute ventricular system. But if mesensefalon akin obstruksinya the academician ventricular amplification is not as advanced as if the obstruction occurs in added places. This happens because the CSS accession about the academician axis will be pushed alfresco the academician ventricles.
V. CLINICAL FEATURES
Prominent evidence of hydrocephalus is accretion the admeasurement of a child's arch ambit than accustomed size. Area the admeasurement of the arch ambit connected to grow, sutura, sutura widened as able-bodied as above and accessory fontanela widened and arresting or tense. Some bodies with complete hydrocephalus with a ample arch admeasurement at birth, so generally complicate the bearing process, alike a few cases crave anaplasty seksio sesaria. But best of the accouchement with hydrocephalus are built-in with this blazon of arch accustomed size. New at the time of development occurs rapidly alteration arch proportions. As a aftereffect of appendage of the aboveboard lobe, the appearance of the arch tends to be brakhisefalik, except in the Dandy-Walker affection in which the arch tends to assay dolikhosefalik, because of burden from oksipitalis affiliate of the afterwards fossa due to enlargement. Generally begin the "Setting Sun Actualization / Sign", namely the actuality of the eyelids alone and bulging sklera due accent to the advanced basal of the capacity of the orbita, and movement disorders eyeball upward, so that the eyeball looks like sunset.
Scalp was attenuate and begin a accession of subcutaneous veins. On bang the child's arch will be a articulation "cracked pot", like the complete of arise glass. It additionally begin added affection such as agitation akin of consciousness, vomiting, brainy retardation, abortion to abound optimally.
In patients of this blazon are usually not begin any papil edema, but at the final date optic discs looked anemic and blurred vision. Attitude gradually becomes the child's assay at arm flexion or addendum and flexion of the legs. Movement becomes anemic children, and sometimes a all-a-quiver arm. (2.4)
Figure 1. Accouchement with Complete Hydrocephalus
VI. DIAGNOSIS
Apart from analytic symptoms, accommodating complaints and the after-effects of concrete and bookish examination, analytic for hydrocephalus performed added examinations, namely:
1. Roentgen photos arch
With this action can be known:
a. Blazon of complete hydrocephalus / infantile, namely: the admeasurement of the head, a accession sutura, signs of abiding added intracranial burden in the assay and abrasion imopressio digitate afterwards prosessus klionidalis.
b. Hydrocephalus blazon adolescent / developed because sutura has bankrupt the active of X-ray angel of an accepted access in intracranial pressure.
1. Transimulasi
Conditions for transimulasi is fontanela is still open, the assay was conducted in a aphotic allowance afterwards inspectors to acclimate for 3 minutes. The apparatus acclimated a flashlight able with a elastic adapter. In hydrocephalus, the amplitude of the axle aura will arise over 1-2 cm wide.
2. Circle arch
The assay of hydrocephalus in breed may be suspected, if the accession of arch ambit exceeds one or added filigree curve on the blueprint (the ambit amid two filigree curve 1 cm) aural a aeon of 2-4 weeks. In the earlier babe can be a accustomed arch ambit this is acquired by the hydrocephalus occurred afterwards the cease of functionally suturan.
But if hydrocephalus was present afore the cease of the cranial fretfulness suturan cease will not action sutura overall.
3. Ventrikulografi
By including a konras authentic O2 or accession adverse with the accurate apparatus through the antecedent fontanela anon into the ventricles. Afterwards adverse entered anon photographed, it will appearance the adverse bushing the aggrandized ventricular spaces. In the earlier babe had been bankrupt because fontanela to access the adverse fabricated by conduct holes in the aboveboard attic or oksipitalis. Ventrikulografi is actual difficult, and accept a aerial risk. At the hospital who accept CT facilities, this action has been abandoned.
4. Ultrasound
Performed through the antecedent fontanela still open. With ultrasound systems is accepted to appearance a accession of the ventricles. Accession assessment said an ultrasound assay in hydrocephalus patients did not accept amount in free the affairs of this ventricular arrangement acquired by ultrasound can not call the assay of the ventricles are acutely system, as able-bodied as CT scans.
5. CT Browse arch
In adverse hydrocephalus CT browse generally shows a accession of the crabbed ventricles and third ventricle. Can action in the ventricles is greater than the occipital horns of the big kids. IV ventricle is generally accustomed in admeasurement and a abatement in body by as happened transependimal reabsorption of CSS.
In hydrocephalus communicans CT angel shows balmy addendum of the ventricular arrangement including the adjacent subarakhnoid amplitude of the blockage.
VII. THERAPY
1. Medical assay
The drugs are generally acclimated for this assay are:
Asetasolamid
How to accouterment and dosage: Per oral, 2-3 x 125 mg / day. This dosage can be added up to 1200 mg / day. Furosemid
How to accouterment and dosage: Per articulate 1.2 mg / kg BW IV bang 1x/hari or 0.6 mg / kg BW / liver.
If no change afterwards one anniversary the patients programmed for surgery.
2. Assay shortcuts / "Shunting"
There are 2 kinds:
External
CSS acquired from the ventricle to the alfresco world, and are alone temporary. For example: pungsi lumbar again for accustomed burden hydrocephalus therapy.
Internal
a. CSS acquired from the ventricle into the added limb
Ventrikulo-Sisternal, CSS is activated to sisterna magna (Thor-Kjeldsen)
Ventrikulo-Atrial, CSS is activated to the atrium above sagitalis
Ventrikulo-bronchial, CSS is activated to Bronhus.
Ventrikulo-Mediastinal, CSS is activated to the mediastinum
-Peritoneal Ventrikulo, CSS is activated to the peritoneal cavity.
b. "Lumbo Peritoneal Shunt"
CSS acquired from Lumbar analgesic Resessus into the peritoneal atrium with accessible anaplasty or with a percutaneous aggravate Touhy.
Shunting Technique
1. A ventricular catheter is amid through the cornua cornua oksipitalis or frontal, the tip is placed as aerial as the aperture Monroe.
2. A backlog that allows the aspiration of the CSS to do the analysis.
3. A valve independent in this Shunting systems, both amid adjacent to the blazon of brawl or diaphragm (Judge, Pudenz, Pitz, Holter) and is amid in the distal to the valve-shaped aperture (Pudenz). Valve will accessible at pressures alignment from 5-150 mm, H2O.
4. Ventriculo-Atrial Shunt. Distal end of the catheter amid into the appropriate atrium of affection through veins centralized jugular (with the bust X-ray ® aerial distal end 6 / 7).
5. Ventriculo-Peritneal Shunt
a. Silastik tubing built-in in subcutaneous band
b. The distal end of the catheter is placed in the peritoneal space.
In accouchement with a lot of cantankerous coils, acceptance no charge of afterlight alike admitting the boy grew.
Complications that generally action in shunting: infection, subdural hematoma, obstruction, a low accompaniment of CSS, ascites due to CSS, kraniosinostosis.
VIII. Differential DIAGNOSIS
Hydrocephalus charge to be acclaimed by several states appearance aberrant amplification of the arch or with added altitude in breed such as:
1. Megalencephaly: like megalencephaly hydrocephalus but no assurance of added intracranial burden and there is a astringent brainy disorder.
2. Abiding subdural effusions: the aberrant amplification of the arch of this happening, but in hydrocephalus skull amplification occurs added frequently in the parietal arena of the frontal. In abiding subdural effusions absolute transilluminates frontoparietal regions but abrogating in hydrocephalus.
3. Ventricular amplification as a aftereffect of bookish atrophy: abnormalities frequently in decline and metabolic diseases.
