Hydrocephalus 1

8/6/2019 Hydrocephalus 1

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HYDROCEPHALUS

ByRatheesh G

1st Year Msc Nursing

CON, KMCH

Anjarakandy


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Definition

Hydrocephalus means abnormal or

excessive accumulation of CSF inthe intracranial cavity.

To understand Hydrocephalus , we

have to take an idea about the

cerebrospinal fluid (CSF) circulation.


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CSF Circulation


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CSF Circulation CSF is a dynamic fluid, its main function

is to keep the internal environment ofCentral Nervous System (C.N.S)constant. It also play a role in mechanical

protection of the brain and spinal cord.

Because CSF is dynamic fluid it issecreted and reabsorbed continuously.CSF is secreted by choroid plexus, the

main bulk of choroid plexus is present inthe Lateral Ventricles, but there ischoroid plexus also in the Third Ventricleand Fourth Ventricle.


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CSF Circulation (Cont.) The rate of formation ofCSF is about 0.3-

0.35 ml/min i.e. it is about 500 cc/dayalthough the constant amount of CSFpresent in the ventricles and subarachnoid

space is about 150 cc. This means thatmost of the amount secreted is reabsorbedagain.

CSF pass from lateral ventricles through

Foramina of Monro to the third ventricle,then throughAqueduct of Sylvius to thefourth ventricle .


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CSF Circulation (Cont.) The fourth ventricle have three aperturesor foramina, One mid-line (Foramen ofMagendi) and two lateral foramina(Foramena of Luschka) (Fig. 2). Throughthese Foramina CSF passes from theventricular system to the subarachnoidspace .

It accumulate first in Cisterna Magna, partof CSF descends downwards around thespinal cord, but the majority passes

upwards through the Tentorial Hiatusover the surface of both cerebralhemispheres to be absorbed by the

Arachnoid villi in the Superiorsagittalsinus SSS .


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Figure 2: CSF Circulation


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CSF Circulation (Cont.)

So, CSF is secreted from the bloodby choroid plexus and reabsorbed inthe blood byArachnoid villi, thisexplains the physiological dynamicfunction of CSF that keeps theinternal environment of CNSconstant.


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Figure 3: CSF Circulation


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ETIOLOGY

INHERITETEDGENITIC

ABNORMALITIES


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ETIOLOGY

DEVELOPMENTALDISORDERS


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at ogenes s oHydrocephalus

Any disturbance in CSF secretion,

circulation and absorption will results in

abnormal accumulation, so thepathogenesis may be :

Obstruction of CSF pathway (main

factor).

Excessive formation of CSF as in case of

choroid plexus papilloma ( rare ).

Decreased absorption as in SSS

thrombosis around arachnoid villi.


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Etiology of CSF pathwayObstruction Congenital anomalies: this is Commonly

seen in the area of Aqueduct, such asforking of Aqueduct, gliosis or obstruction.

N.B. CNS is the commonest system in thebody liable to congenital anomalies.

Posttraumatic or PostHemorrhagic:Subarachnoid hemorrhage, whetherspontaneously (as in Ruptured Intracranialaneurysm or AVM) or after head traumamay result in inflammatory reactions,adhesions, and obstruction ofsubarachnoid s ace and so H droce halus


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Clinical Presentation ofHydrocephalus Clinical picture of Hydrocephalus depends

on the age at the time of presentation.

The skull ofthe infant is malleable andsoft with open sutures and fontanels, so it

can accommodate large amount of CSF

without any increase in the intracranial

pressure.

The skull ofthe adult is rigid, hard, so any

increase in the contents will result in

increase in the Intracranial pressure.


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PostInflammatory or Post-meningitic:

Inflammation of Meninges usually heals byFibrosis and adhesions within thesubarachnoid Spaces, so obstruction ofCSF pathway.

Neoplasm: Any abnormal mass such as

tumor or abscess along the pathway ofCSF may result in obstruction.

N.B: In some cases of Hydrocephalus, wecan not diagnose the underlying etiologicalcause, this is what is called Hydrocephalus

of undetected cause. These cases aremostly due to mild unnoticed Head traumaor sub clinical Meningitis.


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Clinical Presentation ofInfantile Hydrocephalus (Fig.4)

Large sized head or progressive enlargement ofthe size of the head.

The scalp over itw

ell be stretched, shinnyw

ithdilated veins.

The Anterior Fontanel (AF) well be open, wide andbulged. The posterior fontanel may be still open.

Sun set appearance of the eyes.

Disproportion between the size of the head and

size of the face. Disproportion between the size of the head and

size of the body.

In advanced cases, delayed development of thenormal milestones and Mental retardation may be


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Figure 4: InfantileHydrocephalus


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Investigations ofHydrocephalus Wewell mention here all available investigations

for Hydrocephalus, but it is not necessary to do

all the investigations for every patient. Ultrasonography (US):

It is the first investigatory methods for Infantilecases, because it needs open AF, It is simple,non invasive and cheep. It is the method ofchoice to diagnose intrauterine cases.

Plain x-ray skull:

Plain x-ray skull have no role in diagnosis ofinfantile cases. In adults, it may showpathological intracranial calcification, suture


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Investigations ofHydrocephalus (Cont.) Computerized Tomography (CT):

It is main diagnostic tool of

Hydrocephalus, CT confirmsdiagnosis, localizes the site ofobstruction and will show theunderlying cause if present(e.g. Posterior fossa tumor fig. 5 & 6).

Magnetic Resonance Imaging (MRI): MRI have nearly the same diagnostic

value of CT but it is expensive, notavailable at any place, needs long


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Figure 5 & 6: Post. fossatumor with Hydrocephalus


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Management ofHydrocephalus The best line of treatment of

hydrocephalus is to remove the cause of

disease, such as fulguration of choroidplexus papilloma or removal of postfossa tumor.

This is not possible in the majority ofcases i.e. we can not correct congenitalmalformation or remove glioma frombrain stem, so in these cases we divertCSF pathway to overcome site of


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Figure 7 & 8: Shunting inHydrocephalus


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Management ofHydrocephalus (Cont.) Shunt is still the most common method

used to treat hydrocephalus. This is done

by implanting a tube in the lateral ventricle

and the distal end of the tube is put in the

right atrium of the heart through internal

jugular vein (VA shunt),

or pass subcutaneously in front of thechest wall to be implanted in the

peritoneal cavity (VP shunt).


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Management ofHydrocephalus (Cont.) Recently with advancement of endoscopic

surgery we can use special neuro

endoscope to create an opening in thefloor of third ventricle to bypassobstructed Aqueduct and allow CSF topass from Lateral ventricles to the thirdventricle and then to the subarachnoidspace.

This is called Endoscopic ThirdVentriculostomy ( ETV ), this method is anew method, needs special training andequipment and it is not suitable for all


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Hydrocephalus (Cont.) Although shunt is the commonest method

for treatment it has some disadvantages:

Shunt system is expensive. It is a foreign body, so during implantation if

exposed to contamination, this may lead tomeningitis or encephalitis which may be lethaland in such cases shunt should be removed.

It is a narrow tube, so it is liable forobstruction

(either proximal or distal end). It may over drain the CSF so devices must be

selected properly according to the estimatedintra-ventricular pressure of the patients.


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Management ofHydrocephalus (Cont.) Medical treatment have no role in the

management of hydrocephalus, so using

carbonic anhydrase inhibitor( Diamox ),will decrease the rate of secretion of CSFto some extent, but does not relief theobstruction so medical treatment may beused in doubtful cases or if there is

contraindication for surgery such asbronchopneumonia orinfection at the siteof operation.


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SPINA BIFIDABy

Prof. Roshdy El-KhayatProfessor of Neurosurgery,

Vice dean of the Faculty of Medicine,

Assiut University, Egypt.


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Definition

Spina Bifida means abnormal or failure

of fusion of the neural tube.

The Neural tube is formed by

progressive fusion of the edge of Neural

groove. This fusion usually starts in the

dorsal region then extends to the cervical

and cranial part and then progressesdistally.

The last part of Neural tube that fuses is


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Figure 1: Fusion of theneural tube


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Types

There are two main types of SpinaBifida: manifesta and occulta.

Failure of fusion of the neural tubewith failure of fusion of theoverlying osseous, muscular andcutaneous tissue, will result inSpina Bifida Cystica or Manifesta.

Failure of fusion of the neural tubewith intact overlying tissue will


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Spina Bifida Cystica (fig.2) Cystic swelling in the back dating since

birth. It may be present in the back from

cranio-cervical junction to lumosacral

region.

In about 85%of the cases, it presents in

lumbosacral region, 10% in cervical and

5% in the dorsal region. Types of Spina Bifida cystica

(manifesta):


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Hydrocephalus & Spina bifida:Hydrocephalus & Spina bifida:


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Hydrocephalus & Spina bifida:Hydrocephalus & Spina bifida:


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Meningocele It is a cystic swelling in the back dating

since birth.

It contains CSF only. Usually covered with irregular area of

skin and membrane, or may be justmembrane.

There is a cross fluctuation between the

cyst and AF (Anterior Fontanel). Movement Of lower limbs are normal

and sphincters are intact.

It may be associated with Hydrocephalus


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Meningocele (Cont.)

Differential diagnosis of a swelling

in the back since birth:Meningocele.

Lipoma ( soft, slippery edge,

healthy skin ).

Teratoma ( Heterogeneous, intacthealthy skin ).

Haemangioma ( Discoloration,


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Figure 2: Spina bifidacystica


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Meningomyelocele

It has the same characters as

Meningocele.

But it contains Neural tissue, so it is

associated with:

Weakness of one or both lower limbs.

Sphincteric disturbance in the form of

Overflow incontinence of urine, can bediagnosed in infants by observing

continuous dribbling of urine from

external meatus and dullness of su ra


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:Spina bifida


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Meningomyelocele (Cont.)

These neurological deficits inMeningomyelocele is irreversibleand usually can not be correctedby surgery.

So it is very important todifferentiate meningomyelocele

from Meningocele and explains theprognosis for the parents andobtain written consent from them


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Encephalocele:


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Management of Spina BifidaCystica Both types, Meningocele and

Meningomyelocele need excision and

repair but:

Meningocele needs urgent excision and

repair to protect patient from infection or

rupture. Prognosis in such cases is

good. In Meningomyelocele, operation is

elective, many surgeons dont like to


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Spina Bifida Occulta

Means failure of fusion of the posterior

wall of the spinal canal with intact

overlying muscles and skin. So it is

usually occult, but sometimes have

marker on the overlying skin in the form

of: tufts of Hair, abnormal pigmentation

or skin dimple. These are called markeror pointer of Spina Bifida Occulta.

Spina Bifida Occulta is commonly found


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Spina Bifida Occulta(Cont.) Spina Bifida occulta is usually

asymptomatic, and detected accidentally

in plain x-ray lumbosacral region donefor another complaints such as renalcolic or abdominal troubles.

In a minority of cases, spina bifidaocculta causes some troubles foraffected persons because of the

presence of adhesions between thedorsal aspect of the dura and theoverlying fascia and muscles.

These adhesions will restrict the u ward


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Tethered Cord

Tethered cord due to spina bifida

occulta will cause symptoms in adult life

when the traction of the cord reachs

maximum. These symptoms may be:

Hypothesia of root distribution which may

cause trophic ulcer.

Weakness of a group of musclessupplied by affected roots in the form of

weak dorsiflexors of the foot or big toe.

S hincteric disturbance usuall in the


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Tethered Cord (Cont.)

Ifwe have a patients complaing

from one or more of thesesymptoms and plain x-ray spine

shows spina bifida ( Fig. 3 ) we

must be sure that the causative

lesion of these manifestations istethered cord and this can be

known by either Myelogram or MRI


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Figure 3: Spina bifidaocculta (plain x-ray)


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Figure 4: Spina bifidaocculta (Myelogramand MRI)


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Tethered Cord (Cont.) If the cord appears at its normal position,

so the spina bifida occulta is not the

causative lesion andw

e have to searchfor another pathology for thesesymptoms.

If the cord appears in MRI or Myelogrambelow the level of L1 vertebra, thisconfirms that tetherd cord is the

responsible factor and these cases willneed surgical interference to dissectadhesions and freeing the cord. If thisinterference is done early, the recovery


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Spina bifida occulta(Conclusion) Spina bifida occulta is commonly asymptomatic

and detected accidentally on doing x-ray onlumbosacral region.

pointer or marker sometimes presents on theskin such as tufts of hair, abnormal pigmentationor skin dimple.

If spina bifida occulta gives symptoms this iscommonly appears in adult and to blame it as the

causative lesion, we have to confirm this byeither MRI or Myelogram. If the cord is tethered,so it is proved to be the causative lesion andthese cases will need treatment. If the cord


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Hydrocephalus 1

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