8 Hydrocephalus

8/6/2019 8 Hydrocephalus

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HydrocephalusDr Yog Raj Khinchi


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It is t he dila t a t ion of t he ven t ricular sys t em due t oimbalance be tw een produc t ion and absorp t ion of CSF w it hor w it hou t increase in CSF pressure

CSF is produced by choroid plexus by ul t ra fil t ra t ion andac t ive secre t ion . CSF is absorbed in arachnoid villi.

Abou t 20 ml CSF is secre t ed and absorbed in one hourThe t urn over of CSF is 3-4 t imes per day

Defini t ion


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CSF Production

Produced in the ventricles by the choroid plexus by ultrafiltration of the plasma and carbonic anhydrase catalyzedsodium potassium pumpExits the ventricles via the foramina of Magendie andLuschkaEnters subarchnoid space goes around the spinal cord andbrain.CSF is reabsorbed by the arachnoid villi into the saggital veinAdult produces approximately 500 ml a day (20 ml/hr)

CSF volume at one time is approx 150 ml Volume in the ventricles about 25 ml


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V en t ricular Sys t em


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CSF pat hw ayLat eral ven t ricle

Foramen of Monro

3rd ven t ricleAqueduc t of Sylvius

4 t h ven t ricleForamina of Luschka and Magendie

Cist erns

Cerebral and spinal sub arachnoid space(Absorbed via arachnoid villi in t o venous channels)


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Hydrocephalus:No t e t he marked dila t ion of t he cerebral ven t ricles


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Types and E t iologyHydrocephalus resul t s due t o an imbalance be tw een produc t ionand absorp t ion of CSF

Tw o t ypes:

- Communica t ing- Non communica t ing

Communica t ing : No block in pa t hw ay of CSF

Non communica t ing: Block a t any level of ven t ricular sys t em(commonly a t aqueduc t al or foramina of Luschka and Magendie )

Congeni t al or Acquired hydrocephalus


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Causes: Congeni t al hydrocephalus1. In t ra u t erine infec t ion: like rubella, CM V , t oxoplasma

(Inflamma t ion of ven t ricular ependyma, meninges, basal cis t ern)2. In t ra cranial hemorrhage3. Malforma t ions:

(i) Aqueduc t al s t enosis

(ii) Dandy w alker syndrome(pos t erior fossa cys t con t inuous w it h 4 t h ven t ricle)

(iii) Arnold Chiarri malforma t ionType 1 Presen t s in adolescence or adul t lifeNo t associa t ed w it h hydrocephalusHernia t ion of cerebellar t onsils in t o cervical canalType 2 Symp t oma t ic in infancyProgressive hydrocephalus + myelomeningoceleHernia t ion of cerebellum pons and medulla in t o cervicalcana l

4. Congeni t al midline t umours obs t ruc t ing CSF flow


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Causes: Acquired hydrocephalus

Tuberculous and pyogenic meningi t is

Pos t -in t raven t ricular hemorrhage

Pos t erior fossa t umours

AV malforma t ions, in t racranial hemorrhage


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O t her t ypes of hydrocephalus:

Pan ven t ricular hydrocephalus Dila t a t ion of la t eral, 3 rd and 4 t h ven t riclesDouble compar t men t hydrocephalus Also called t rapped 4 t h ven t ricle There is aqueduc t al s t enosis along w it h obli t era t ion of

t he ou t le t of 4 t h ven t ricleUnila t eral hydrocephalus Due t o compression of opposi t e la t eral ven t ricle O bs t ruc t ion t o foramen of monro

Hemi parenchymal atrophyHydrocephalus ex vacuo

V en t ricular dila t a t ion associa t ed w it h cerebral a t rophy-CSF pressure normal


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Pa t hologyUn even dila t a t ion of ven t ricles

Ependymal lining of ven t ricles disrup t ed Peri ven t ricular ooze

Sub ependymal oedema occurs

Whi t e ma tt er compressed

Lat er Cor t ical a t rophy

Process reversible on early t rea t men t


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Cut sec t ionsa t various

levelssho w ingdila t edven t ricles


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Clinical fea t ures

Enlarging head size and delayed closure of fon t anelles andsu t ures

Symp t omsHeadache, nausea, vomi t ing, personali t y and behaviorchanges such as irri t abili t y, head banging, apa t hy,dro w siness


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Papilloedema, pyramidal t rac t signs cranial nerve palsies,abnormals skull con t our, prominen t scalp veins

Sunse t sign- sclera above cornea becomes visible

Limbs become spas t ic because of s t re t ching of cor t ical fibres

Dist or t ion of brains t em bradycardia, sys t emic hyper t ension,al t ered respira t ory ra t e

Large head size a t bir t h causes difficul t y in labour. There may

be associated malforma

tions

Signs


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Sunse t Sign


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Diagnosis

Accura t e serial recording of head circumference + suppor t iveevidence by USG early diagnosis

If head circumference in t he firs t 3 mon t hs of life of over 1cm every for t nigh t should arouse suspicion of hydrocephalus

Persis t en t w idening of squamo parie t al su t ures, is no t

physiological


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Diagnosis...

Cranial USG and CT helps evalua t e ven t ricular size, cor t icalman t le, peri ven t ricular ooze and e t iology of hydrocephalusX ray

Sut ural separa t ion Erosion of pos t erior clinoid process Silver bea t en appearance ( in convolu t ional markings )


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CT Scan sho w ing Dila t ed V en t ricles


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CT head sho w ing dila t ed ven t ricles:


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CT head sho w ing dila t ed ven t ricles:


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T1-w eigh t ed axial MR I sho w s markedlydila t ed la t eral ven t ricles w it h lo w signal CSF.

T2-w eigh t ed axial MR I sho w s high signal CSFof t he markedly dila t ed la t eral ven t ricles.


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Differen t ial diagnosis of a large head

Megalencephaly No signs of ICT V en t ricles normal Eg. Hurlers Disease, Taysachs disease, Me t achroma t ic

Leukodys t rophyChronic subdural haema t oma Mos t ly loca t ed in parie t al area w it hou t prominen t scalp

veins or sunse t signs

O t her causes Hydrencephaly, ricke t s , achondroplasia, hemoly t ic

anemia, macrocephaly (familial)


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Trea t men t ...

Shun t sV en t riculoa t rialV en t riculo azygousV en t riculo peri t onealTheco peri t onealV en t riculo pleuralV en t riculo t horacic duc t al


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Complica t ions of shun t

Blockage, infec t ion, peri t oni t is, endocardi t is, arrhy t hmias

Shun t should be kep t for en t ire life

Revise t he shun t using a longer t ube as t he child gro w s

Shun t revision if t here is infec t ion


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Prognosis

Na t ural his t ory of un t rea t ed cases50% die, 50% surviveIn survivors 50% have handicaps, 50% normalin t elligence

Prognosis depends on cause of hydrocephalus and no t ont he size of cor t ical man t le a t diagnosis

Congeni t al hydrocephalus w it h spina bifida has poorprognosis

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8 Hydrocephalus