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Review Article

Perinatal ventriculomegaly

Running title: Fetal and neonatal cerebral ventriculomegaly

Sahar M.A. Hassanein a,*, Husein Moharram b, Ahmed Mohamed Monib c, Ahmed R.M.R.A. Ramy d and alid Abdel !hany b

a

Department of Pediatrics, Ain Shams University Hospital, Cairo, Egypt Department of Neurosurgery, Ain Shams University Hospital, Cairo, Egypt Department of Radiodiagnosis, Ain Shams University Hospital, Cairo, Egypt Department of !stetrics and "ynecology, Ain Shams University Hospital, Cairo, Egypt

b

c

d

*

"orres#ondence: Sahar M.A. Hassanein

$e#artment o% Pediatrics Ain Shams &niversity Hos#ital, "airo, 'gy#t '(mail address: saharhassanein)yahoo.com *el.: +,-+.-/.01.2 Fa3: 4+,+5 +6/+,+.72 Received: 8anuary ,7, +,,/ Revised: 8une ,7, +,,/ Acce#ted: 8une -1, +,,/

+

Abstract. 9entriculomegaly is an increase in cerebral ventricular dimensions. :t could be secondary to increased cerebros#inal %luid #ressure in the ventricular cavity 4hydroce#halus5, or to a lac; o% brain #arenchyma due to atro#hic involution or arrested develo#ment. 9entriculomegaly causes diagnostic, thera#eutic, and #rognostic dilemma %or obstetricians, neonatologists, and neurosurgeon. Mild %etal cerebral ventriculomegaly or borderline ventriculomegaly is de%ined as an a3ial diameter < -, mm across the atrium o% the #osterior or anterior horn o% lateral ventricles at any gestation. Moderate ventriculomegaly is atrial diameter larger than -= mm2 residual corte3 larger than two mm in diameter and severe 4atrial diameter larger than -= mm residual corte3 smaller than two mm in diameter5. Sonogra#hic demonstration o% adducted thumbs in combination with ventriculomegaly and other intracranial abnormalities should #rom#t the diagnosis o% >(lin;ed hydroce#halus s#ectrum. Fetal brain magnetic resonance imaging, echocardiogra#hy, abdominal ultrasonogra#hy, ;aryoty#e, and serologic tests %or congenital in%ections are indicated. ?eonatal ventriculomegaly could be @ust due to increased width o% cerebral ventricles or with increased cerebros#inal %luid volume i.e. hydroce#halus. Hydroce#halus could be obstructive 4non( communicating5 hydroce#halus or non(obstructive 4communicating5. Also, hydroce#halus course could be active 4decom#ensated or #rogressive5 or com#ensated 4arrested5. :ntraventricular hemorrhage is the most common cause o% acAuired hydroce#halus 4#ost( hemorrhagic hydroce#halus5 in #reterm neonates. Progressive hydroce#halus is associated with head circum%erence, crossing centile lines or e3#anding at over -.=(+ mmBday, a tense anterior %ontanel and se#aration o% the cranial sutures, a#nea, vomiting, and abnormal #osture. Prevention and management o% #ost(hemorrhagic hydroce#halus is very challenging. Shunt o#eration is the de%initive treatment o% #rogressive hydroce#halus due to any cause. Cey words: AAueduct stenosis, "?S mal%ormations, %etal, hydroce#halus, intraventricular

. hemorrhage, meningomyelocele, neonatal, #erinatal, #ost(hemorrhagic hydroce#halus, #ost( hemorrhagic ventricular dilatation, ventriculomegaly

At. hile at term. However.s or later. +. but as the %etus a##roaches term this ratio decreases.e.th #ostmenstrual wee. *he Aualitative method de#ends on suggestive changes in the sha#e o% the lateral ventricles and the a##earance and the mobility o% the choroid #le3us 4dangling choroid #le3us sign5. in the second trimester the ratio o% the lateral ventricles is higher com#ared to the brain #arenchyma..(6. *he Auantitative method is done by measuring the ventricular siIe. any cases o% 9M does not become a##arent until -6th to -/th #ostmenstrual wee.5.e. or it may be the result o% #assive enlargement caused by atro#hy o% the brain #arenchyma. 9M means enlargement o% the ventricles. DH"PE. %etal age must be assessed when loo. *he incidence o% congenital cerebral lateral 9M ranges between .. births 4...s o% gestation the lateral ventricular sha#e is more G#lum#G but still within the normal limits %or this age. which may be caused by increased intraventricular #ressure secondary to obstruction o% cerebros#inal %luid 4"SF5 %low i. As.ing %or the #resence o% 9M 4=5. Huantitative and Aualitative methods can be used to assess the %etal brain. Introduction *he term DventriculomegalyE 49M5 is o%ten used interchangeably with hydroce#halus 4H"P5.1 1.F o% %etuses with enlarged lateral cerebral ventricles 415.. Also. to -.= in -. Fetal VM "ongenital lateral cerebral 9M is among the most common brain abnormalities that can be detected by routine ultrasonogra#hy 4&S5 4+5. :n s#ite o% the advances in &S. 2. 9M could be detected antenatal by the obstetrician during routine maternal %ollow u# 4%etal 9M5 or diagnosed a%ter delivery 4neonatal 9M5 4-5. *here%ore. *he commonly used normal . :solated 9M accounts %or . the early diagnosis o% %etal 9M or H"P remains a diagnostic challenge. the normal %etal lateral ventricle a##ears slit li.

. #$%$%$ &ild or !orderline '& *he widely acce#ted de%inition is an atrial width o% -. Most de%ine them as moderate to severe 9M 4Fig.s o% gestation 475. :n addition.= values %or %etal lateral ventricles were generated using measurements obtained transabdominally 4*A5 in the a3ial #lane. mm across the atrium o% the #osterior or anterior horn o% lateral ventricles at any gestation through *A scans. or *A by multi(#lanar imaging o% the brain 4three(dimensional &S ..5. Kther #ro#osed de%initions include an atrial width o% -.$5.(-= mm on the transverse #lane.6 mm 4mean J S$5 %rom -1th to .6 J . *he volume o% the %etal brain can be obtained transvaginally.5. 4-=5 is ma3imum width o% the atrium o% the lateral ventricle at least -= mm but there is still more than three mm o% cortical mantle. Another de%inition by Levine et al. #$%$ Definition Although mild or borderline 9M is well de%ined and got an acce#ted norms among radiologists. -5. the volume can be saved and reviewed later 465. :solated Mild 9M is 9M in the absence o% other demonstrable central nervous system 4"?S5 anomalies 4/5.(-+ mm 405 and a distance greater than %our mm between the medial wall o% the ventricle and the glomus o% the choroid #le3us 4-. *he atrial diameter is constant at 7. there is no acce#ted de%initions %or grading moderate or severe 9M. Fetal cerebral 9M is de%ined as an a3ial diameter < -. #$%$#$ &oderate(to(severe or overt '& *here is no acce#ted consensus about either moderate or severe 9M as se#arate grading. #$%$)$ &oderate :t is atrial diameter < -= mm and residual corte3 < two mm in diameter 4-15./th wee. Most de%ine a moderate to severe or overt 9M as ventricles <-= mm wide 4--(-.

(lin-ed HCP spectrum . including hy#o#lastic or %le3ed adducted thumbs. and H"P5.neural cell adhesion molecule5. Retardation. Adducted thumbs. #$#$ Etiology 9M can arise %rom agenesis o% the cor#us callosum.6 #$%$*$ Severe :t 9M is atrial diameter < -= mm and residual corte3 < + mm in diameter 4-15.ed H"P s#ectrum. and Adducted thumbs5. *hese related diseases are: M "ongenital stenosis o% the aAueduct o% Sylvius: "ongenital H"P and resultant macroce#haly due to stenosis o% the aAueduct o% Sylvius may occur in isolation but is %reAuently associated with other %eatures. A#hasia. *here can be signi%icant #henoty#ic variability within %amilies. Mutations in this gene are also res#onsible %or other syndromes with clinical overla# that are %reAuently re%erred to as the >(lin. *hese related neurological syndromes are caused by mutations in the gene at >A+/ encoding %or the L-"AM 4L.-75. 4-=5 is ma3imum width o% the atrium o% the lateral ventricle at least -= mm but there is no more than three mm o% cortical mantle. S#astic #ara#legia. M MASA Syndrome 4Mental retardation. cerebral vascular anomalies. cerebral maldevelo#ment or destruction.ed s#astic #ara#legia 4SP -5 #atients are mentally retarded and have s#astic #ara#legia. Another de%inition by Levine et al. with some males being severely a%%ected and diagnosed already during #regnancy. #$#$%$ +nherited #$#$%$%$ . Shu%%ling gait. or an obstruction within the ventricular system 4-6. M >(lin. . while others may have no macroce#haly and long survival 4-/5. M "RASH Syndrome 4"or#us callosum agenesisBhy#o#lasia./% Disease0 :t com#rises a##ro3imately =F o% all cases.

and this can be a use%ul sign to %ollow over time 4/(-+5.7 M MR("* syndrome 4>(lin. rubella5 have been strongly associated with increased H"P #revalence. 9M is considered asymmetric. hether 9M is isolated or associated with other mal%ormation. M Some %orms o% >(lin. *he #renatal detection o% such abnormalities is critical.ed agenesis o% the cor#us callosum 4-05. Starting at around -. the transverse diameter o% the atria is stable in siIe and is usually less than -.ed Mental Retardation("las#ed *humb5. *hey are also o% great value %or #rognostication and obstetric management.(-= wee. Although &S is the #rinci#al techniAue %or screening the %etal brain. cytomegalovirus. *he transverse diameter o% the atria is used as a standard. Fetal cranial &S is used to determine the degree o% 9M. #$#$#$ Ac1uired "ongenital in%ections caused by maternal #renatal in%ection as 4to3o#lasmosis. it can be limited in detecting abnormalities o% the brain #arenchyma.. mm.+-5. Se#aration o% the choroid #le3us %rom the medial wall o% the lateral ventricle is used to diagnose 9M i% < . Fetal MR: can detect additional sonogra#hically occult "?S abnormalities in u# to 1. #$)$ Diagnosis Fetal cranial &S and magnetic resonance imaging 4MR:5 are im#ortant tools %or the diagnosis and %ollow u# o% 9M. with an average o% about 7. Fetal brain MR: should be recommended because the #rognosis o% %etal 9M is related to the #resence o% additional abnormalities. :% the di%%erence between the right and le%t sides is greater than two mm.6 mm.F o% cases o% %etal 9M 4++5. :% . or 1 mm. *eratogenic and neo#lastic causes can lead to 9M due to cerebral mal%ormations or H"P 4+. even when the ventricular measurement is borderline. sy#hilis. non(#rogressive or #rogressive will a%%ect the management and #rognosis o% 9M 4Figs -(=5.s o% gestation.FN=.

-5. cerebellar dys#lasia.ed H"P s#ectrum should be in%ormed that a normal mid trimester &S does not rule out this condition 4-75.ed H"P s#ectrum should be o%%ered $?A analysis. because sometimes a generic diagnosis o% congenital H"P may hinder a more com#le3 anomaly with signi%icant genetic im#lications. $andy( . the #atient may undergo a %etal brain MR: to determine the severity o% the %inding and can detect associated brain mal%ormation 4+. 9M may develo# only in late gestation or a%ter birth.ed H"P s#ectrum. intraventricular hemorrhage 4:9H5.. %or %etal cerebral 9M 4because o% a #reviously a%%ected child. and #ontocerebellar dys#lasia and destructive abnormalities. al. #$*$ !stetric management Multi#lanar e3amination o% the %etal brain with a high(resolution vaginal #robe should be done %or #atients at ris. cortical mal%ormations. Caryoty#ing should be done %or %etuses with associated anomalies as they are at greater ris. multicystic ence#halomalacia. #orence#haly.er( arburg syndrome. such as agenesis o% the cor#us callosum. "ou#les with a #reviously a%%ected child should receive genetic counseling. 65. *he #atients at ris. #articularly with the >(lin. Sonogra#hically occult %indings include develo#mental abnormalities. as the recurrence rate is high and mid trimester &S is %reAuently unsuccess%ul 4+/5. and sube#endymal hemorrhage 4+6.ed H"P s#ectrum could be done by the #resence o% adducted thumbs in combination with 9M and other intracranial abnormalities. *ermination o% #regnancy could be an o#tion %or severe 9M associated with other structural mal%ormations such as s#ina bi%ida will usually carry a #oor #rognosis 4.(+=5.. *he diagnosis o% >(lin./ 9M is detected on ultrasound. #eriventricular nodular heteroto#ia. For e3am#le. A %lowchart %or management o% %etal cerebral 9M is constructed 4Fig. #atients at ris. %or >(lin. such as #eriventricular leu.5.omalacia. #artial agenesis o% the se#tum #ellucidum. %or >(lin. or because o% maternal in%ections5.+75. %or an underlying chromosomal abnormality 4+0.

/5. in%ormation should be collected Auic. the #renatal ..75.6.N1F o% cases.. Fetuses with a##arently isolated 9M #resent a di%%icult grou#.15.F ris. :n the absence o% a chromosomal abnormality or any structural mal%ormation there remains the #ossibility o% both death and handica#. Surgical re#air to %etuses with meningomyelocele in(utero has been #ro#osed as a way .6F.5. *ermination o% #regnancy with stable %etal 9M. :t is an o#tion %or treating %etuses with isolated #rogressive 9M to im#rove brain develo#ment. most o%ten trisomy +. Ra#id . although in(utero treatment with ventriculoamniotic shunts has not led to im#roved #erinatal outcomes 4. 9M due to aAueductal stenosis 4H"P5 is traditionally detected and then treated a%ter birth with a shunt #rocedure. Caryoty#ing a%ter termination has a ris. 'ven i% termination o% #regnancy is arranged. :% termination o% the #regnancy is considered.er mal%ormation has an /.+5.0 al. o% %ailure o% cell culture 4. o% handica# is between 0F and .=5.4. Caryoty#ing will identi%y chromosomal anomalies in .ing the diagnosis o% trisomy. Fetal MR: should be #er%ormed be%ore in(utero surgery. is a di%%icult and com#le3 issue 4. 9entriculo(amniotic shunt 4the #lacement o% a tube between the %etal ventricular system and the amniotic cavity to reduce #ressure5 ( the results o% %etal surgery %or isolated #rogressive 9M are not encouraging. o% develo#mental delay in survivors by 1 years o% age 4.ly so that the #arents can reach a decision. #$2$ 3etal surgery *he treatment o% 9M in(utero can be very challenging.aryoty#e o%%ers the best chance o% ma. *heoretically decom#ressing the ventricles may #revent adverse e%%ects on the develo#ing brain.aryoty#ing %or amniocentesis using Auantitative %luorescent #olymerase chain reaction will give a result within 1/ hours 4. where the ris.

:n%ants with a #renatal diagnosis o% mild 9M have abnormal neurodevelo#ment in -. as most o% them do not have macrocrania. 'lective cesarean section is indicated i% there is ce#halo( #elvic dis#ro#ortion 4165.1/5.F to .5. &nilateral mild 9M carries a %avorable #rognosis when isolated 410. #ercent.F o% males5. etiology. even when isolated. #$5$ Recurrence ris"ongenital 9M is mostly multi%actorial e3ce#t %or >(lin. #$4$ Planning delivery A trial o% labor is indicated in most in%ants with 9M and verte3 #resentation. *he #ediatric team should be in%ormed well in advance o% delivery. and using cesarean section %or obstetrical indications only are general recommendations. o% 1F.0(1=5. and ventricular measurement 4+0.ed H"P 4recurrence ris. :t can be reserved %or babies with Auestionable viability to allow vaginal delivery 416. Families with a #reviously a%%ected child have a recurrence ris. avoiding ce#halocentesis unless non(viable baby. long(term e%%ects on brain %unction have not been determined 4. and a de%initive #lan should be available %or the obstetric and neonatal team. Neonatal VM )$%$ Definitions . Although reduction in hindbrain herniation and reduction in shunt(de#endent H"P has been re#orted. "e#halocentesis is indeed associated with a #erinatal mortality in e3cess o% 0.-. 9M associated with abnormal %indings and structural mal%ormations. will o%ten carry a #oor #rognosis %rom disability to death.=. to im#rove neurological outcomes. $elivery is better to be delayed until %etal lung maturity is documented. "e#halocentesis can be done #rior to delivery to reduce the cranial siIe and allow %or vaginal delivery. =. 3.175.6F de#endent on associated anomalies.

ed within the ventricular system. *rue H"P must be distinguished %rom transient 9M. "riteria %or diagnosing arrested or com#ensated H"P are near normal ventricular siIe. :t could be classi%ied according to the timing into congenital or acAuired. ?on(obstructive or communicating H"P occurs when the "SF leaving the %ourth ventricle is restricted in its %low over the sur%ace o% the brain. it could be classi%ied according to the disease course into #rogressive 4active or decom#ensated H"P5 and stationary course 4arrested or com#ensated H"P5. hile. or i% the sites o% absor#tion are not %unctioning adeAuately 4=-5. H"P has many de%initions and ty#es. which occurs secondarily to the loss o% cerebral tissue either because o% the destruction. Arrested H"P or 4com#ensated H"P5 is that the siIe o% the ventricle remained the same %rom one ultrasound to the ne3t. "ongenital H"P means that the condition e3isted be%ore birth. Kbstructive or non(obstructive H"P can be a congenital or an acAuired condition 4=+5.-9M in the neonatal #eriod could be either due to H"P or decreased #arenchymal volume and mal%ormations. %or a variety o% reasons. . and static 9M. and to level o% "SF circulation abnormality into obstructive or non(obstructive. normal head growth curve and normal #sychomotor develo#ment 4==5. o% cerebral white matter %ollowing :9H 4=75.=15. Also. such as trauma. Progressive H"P is the ventricle increases in siIe %rom one ultrasound to the ne3t mani%ested with sym#toms and signs o% increased intracranial tension and #rogressive head e3#ansion. scar tissue %ormation. Kbstructive or non(communicating H"P occurs when "SF %low is bloc.s.. #ost hemorrhagic. or "?S in%ection 4=. which is common %ollowing :9H but resolves com#letely within %our wee. or the %ailure o% develo#ment. acAuired H"P develo#s a%ter birth. An arrested H"P may become active or #rogressive and a #rogressive H"P may arrest or com#ensate 4=65.

$andy al..F o% H"P cases. Kther causes associated with congenital H"P are intrauterine in%ections. #er -. and tumors 46-5.-+ )$#$ 'tiology "ongenital H"P has an estimated incidence o% about three to %our #er -.. "hiari ty#e :: is the most common ty#e and almost always associated with a myelomeningocele and H"P 46+5. Rare causes o% H"P in the neonatal #eriod include arachnoid cysts. congenital choroid #le3us #a#illomas. :n the neonatal #eriod. and sy#hilis 4=0. Although commonly recogniIed antenatal or at birth.6.5. live births 4=/5. "ongenital aAuaductal stenosis occurs in .. *he "hiari ty#e ::: is herniation o% the cerebellum through the %oramen magnum and an associated cervical s#ina bi%ida. *hree ty#es were described de#ending on the degree o% herniation. the disorder may have an insidious onset.er mal%ormation is a less %reAuent cause o% congenital H"P.. Arterio(venous mal%ormations o% the great vein o% !alen or the straight sinus may also #resent in this #eriod and may cause H"P secondary to bloc. cytomegalovirus. and should be considered in the di%%erential diagnosis o% ... es#ecially to3o#lasmosis.age or ru#ture. "hiari mal%ormations and aAueductal stenosis are the most common causes o% congenital H"P due to structural de%ects. "hiari ty#e : is herniation o% the cerebellar vermis or tonsils through the %oramen.= to -. which may subseAuently lead to H"P. Premature in%ants are #articularly susce#tible to :9H. live births. :n ty#e ::. )$)$ &echanism of ventriculomegaly "auses and mechanisms o% neonatal 9M and H"P are summariIed in *able -. the %ourth ventricle and lower medulla are also herniated. accounts %or a##ro3imately +. )$)$%$ Congenital "hiari mal%ormation is a set o% congenital anomalies o% the hindbrain where there is a downward herniation o% the brainstem and cerebellum through the %oramen magnum. acAuired causes o% H"P include #erinatal in%ections and intracranial bleeding secondary to trauma or ano3ia. rubella.

:t occurs in a##ro3imately . live births.er mal%ormations. Oleeding o% these vessels has been classi%ied into %our grades. !rade : is hemorrhage only within the germinal matri3. :t is due to #artial or com#lete agenesis o% the cerebellar vermis... *he diagnosis o% H"P may be delayed until adolescence or adulthood in some cases 46-5. which leads to obstruction o% "SF out%low through the %oramina o% the %ourth ventricle.6-5. H"P at any age. !rade :9 is :9H with ventricular dilatation and s#read o% bleeding into the surrounding brain #arenchyma. !rade :: hemorrhage e3tends %rom the matri3 into the ventricles. but without ventricular dilatation. )$)$#$ Ac1uired HCP AcAuired aAueductal stenosis due to gliosis. can result %rom destruction o% e#endymal cells a%ter a hemorrhagic or in%ectious #rocess 4to3o#lasmosis. cytomegalovirus and mum#s ence#halitis5 4=05. *he $andy( al. Post(hemorrhagic ventricular dilation has high morbidity and mortality 46. . !rade ::: is :9H with resultant ventricular dilatation. Although the de%ect is #resent at birth. H"P will be diagnosed by one year o% age in a##ro3imately /. :9H usually occurs in low birth weight in%ants within 7+ hours o% delivery 4615. and is associated with less than %ive #ercent o% all cases o% H"P 4=/..er mal%ormation is a cystic dilatation o% the %ourth ventricle. non(communicating H"P. Kne %orm o% aAueductal stenosis. :9H is becoming an increasingly im#ortant cause o% H"P secondary to the increased survival o% e3tremely #reterm in%ants. associated with the syndrome o% >(lin. :9H is the result o% vascular instability o% cerebral vessels in the germinal matri3 at the level o% the head o% the caudate nucleus in the #remature in%ant. which is res#onsible %or the #roduction o% s#eci%ic neuronal cell adhesion molecules 46-5.F o% all $andy( al.#er . is caused by a mutation o% the >(lin.-.5.ed recessive Lgene.. AAueductal stenosis results %rom narrowing o% the aAueduct o% Sylvius and leads to obstructive..ed H"P.

raised intracranial #ressure 4:"P5. wee. )$2$ &anagement Management o% neonatal 9M de#ends on the etiology. associated mal%ormations. )$*$ Diagnosis )$*$%$ Neuroradiologic studies "ranial &S remains the main stay as bedside diagnosis o% :9H in #reterm neonates.s to months a%ter :9H. %ree radical generation. and diagnosis o% #eriventricular leu. and in%lammation. :ntraventricular blood and ventricular e3#ansion adversely a%%ects the immature #eriventricular white matter by a variety o% mechanisms. A non(contrast com#uted tomogra#hy 4"*5 should be done to any ence#halo#athic term in%ant with birth trauma. including #hysical distortion. Permanent H"P is induced by the inward migration o% %ibroblasts and collagen de#osition in the "SF #athways 46=5. %or evidence o% hy#o3ic(ischemic in@ury. i% the "* is negative %or hemorrhage 4605.s #ostmenstrual age. *he brea.sP gestation should receive screening cranial &S at age 7 to -1 days and again at . which is a ma@or cause o% cerebral #alsy in term in%ants. $etection o% :9Hs signi%icantly alters clinical care. and .. All #reterm neonates born at less than .-1 Post hemorrhagic H"P 4PHH5 is de%ined as #rogressive 9M caused by disturbances in "SF %low or absor#tion %ollowing :9H. PHH may occur immediately as a result o% multi#le blood clots obstructing the ventricular system or channels o% "SF reabsor#tion initially. $i%%use %ibrosis o% the le#tomeninges leads to communicating H"P within wee. low hematocrit or coagulo#athy to detect hemorrhage. Kbstruction to "SF circulation causes obstructive H"P 467. MR: should be #er%ormed when the in%ant is between + and / days o% age to loo.6/5.oence#halo#athy or low( #ressure 9M alters the #rognosis and outcome.down #roducts o% blood lead to chronic obliterative arachnoiditis o% the basal cisterns and de#osition o% e3tracellular matri3 #roteins in the %oramina o% the %ourth ventricle and the subarachnoid s#ace 4665.6 to 1. wee.

Also acetaIolamide and %urosemide can be used 47. *his includes bulging %ontanel. AcetaIolamide 4-.g. :n%ants with mild or moderate 9M and head circum%erence within the re%erence range may not reAuire initial treatment. some consider e3#ansion o% head by -.= mmBday as e3cessive 47+.gB#er day5 reduces "SF #roduction by =.1 cm over 7 days5. increased irritability. *he . Medications that reduce :"P such as mannitol may be used %or cases o% ra#idly #rogressive H"P while awaiting surgery. -. deteriorating neurological status..-= whether it is an increase in ventricular width without increase in "SF volume or it is H"P. and monitoring o% the head circum%erence and re#eat &S. Shunt com#lications are more common i% done early 47-5. "*. andBor the use o% acetaIolamide and %urosemide to reduce "SF #roduction can be done awaiting 9P(shunt %or ra#idly #rogressive H"P 47=. "SF drainage by re#eated lumbar or ventricular ta##ing. due to the #resence o% large amount o% blood and #rotein level in the "SF together with the small siIe and instability o% the #reemies ma. *reatment o% PHH is more di%%icult than other ty#es o% H"P. :n these in%ants %ollow u# %or the %irst %ew months o% li%e. :% the diagnosis is H"P.7.F. ?ormal head growth at this age is a##ro3imately mm #er day 4715. Knce the underlying etiology has been diagnosed. :n%ants with a clinical #icture o% active H"P and signi%icant 9M reAuire treatment early in li%e. MR: are hel#%ul to decide whether shunting is needed or not 4605. which allows %or diversion o% "SF and ventricular decom#ression. it should be assessed whether it is com#ensated or active H"P 4Figs 7 and /5. absent cerebral diastolic velocity 4not e3#lained by a #atent ductus arteriosus5 or e3cessive head e3#ansion o% + mmBday 4e.es an early 9P shunt o#eration contraindicated.5. the main thera#y %or #rogressive H"P is a shunt #rocedure. mgB.765.5.

*he #otential to3ic e%%ects o% acetaIolamide on myelination should be considered be%ore the initiation o% treatment.g over -.g %or =(7 days. a ventricular reservoir is indicated. :t includes a catheter inserted in the ventricle o% the brain. subdural hygroma and liAuor %istula 4/.(-= mLB. A clinical trial demonstrated the use o% these drugs to be ine%%ective 4775. a one(way valve that allows . )$2$%$ /um!ar and ventricular taps Lumbar #uncture 4LP5 4%or communicating H"P5 or trans(%ontanel ventricular ta# 4%or non(communicating or %ailed LP to reduce ventricular siIe or :"P5 is carried out with the ob@ective o% removing -. min 47/5.in necrosis. )$2$#$ Su!cutaneous reservoir :ntermittent ta#ing through a subcutaneous reservoir is a %reAuently used o#tion. )$2$)$ E6ternal ventricular drainage '3ternal ventricular drainage can be #er%ormed through a shunt inserted into the dilated anterior horn o% the right lateral ventricle. :% the LP %ails to drain enough to normaliIe head growth to Q+ mmBday. shunt in%ection..(-= mLB.5. Failure o% LP may be associated with obstruction o% "SF #athways by blood clots and the change o% ty#e %rom communicating H"P to obstructive ty#e 47=(705. *he amount o% "SF removed can be ad@usted by elevating or lowering the drainage system 47/. *he ma@or com#lications o% subcutaneous reservoir are s.-6 combination o% acetaIolamide and %urosamide reduces "SF #roduction by -. )$2$*$ Surgical therapy of HCP 9entricular shunt is an arti%icial device2 made mostly o% #lastic 4some #arts may be metal5.(+. ventriculitis. *he #ro3imal end o% the catheter is tunneled subcutaneously and is connected to the drainage system which can continuously remove "SF by -. ?eonates on acetaIolamide should have serial renal &S because o% the #ossibility o% ne#hrocalcinosis./-5.F.

:n children with o#en myelomeningocele. another ty#e o% surgical shunt may have to be considered.-7 the unidirectional %low o% "SF out o% the brain. . simultaneous shunting and closure o% myelomeningocele should be #er%ormed. and i% #resent./65. :t a##ears to #rotect #atients %rom "SF lea. "SF testing %or in%ection should be #er%ormed. ta##ing the reservoir continues until the abdomen normaliIes.g but could be lower in individual cases5. :n such circumstances. &sually. and they allow %low o% "SF once that #reset #ressure has been reached. :n current #ractice 9P shunt is most commonly used 4/+5. and a distal catheter that drains the "SF to an e3tracranial location in the body. "SF in%ection may have already ta. simulating the normal %low o% "SF.= gBL is obtained and the in%ant has acce#table weight. Kther sites %or insertion %or rare di%%icult cases with coe3isting abdominal #roblems can be used. till control o% "SF in%ection and a shunt can be inserted 4/7(/05. e3ternal ventricular drainage should be em#loyed %or 7(-. *he %low regulating valves are more #rone to valve obstruction 4/. the gall bladder. Shunt insertion %or PHH could not be done until sterile "SF with no cells and #rotein Q-. and it im#roves the chances %or better develo#ment by reducing intracranial hy#ertension early 4/=. 'ssentially two ty#es o% shunts e3ist: Pressure(regulating shunt is designed to maintain a di%%erence o% #ressure between their inlet and outlet. a low(#ressure valve system is used in shunting babies with PHH 476. %rom the s#inal wound. such as the right atrium. %low regulating shunts allow a constant %low o% "SF.en #lace. days together with antibiotic treatment.7/(/-5. :% there is intra(abdominal #athology such as necrotiIing enterocolitis. or the bladder. *he most #re%erred distal site remains the #eritoneum. which can lead to shunt in%ection./15. i% %easible. the ureter. *he di%%erential #ressure valves are more #rone to cause over drainage com#lications. hile.. which contraindicates a 9P shunt.= . :% closure o% the de%ect has been delayed. 9ery rarely. 4*his weight is usually +.

and it is the #rocedure o% choice in this subgrou# o% #atients. *he most common time %or shunt %ailure to occur is within 6 months o% surgery.-/ S#ina bi%ida reAuires %ollow(u# %or li%e to detect and treat #roblems associated with it. "auses o% shunt mal%unction are obstruction. )$2$*$%$ Shunt complications Shunt mal%unction is a %airly common occurrence with a one(year %ailure rate o% . Higher rates o% %ailure have been described in younger #atient #o#ulations with the most signi%icant ris. occurring in #atients younger than si3 months o% age at the time o% im#lantation.F when used in aAueduct stenosis.0. Mortality rates have been reduced to less than =F in ten years a%ter shunt #lacement . es#ecially Staphylococcus epidermidis. sterility o% the "SF should be con%irmed %irst by culture #rior to surgery 40+(015. :n%ection rates vary %rom -(-.F. :n%ections usually #resents about two months a%ter shunt insertion.F 40+5. :% shunt revision is necessary. in%ection. and over(drainage 40+.5. *he most common causative agent is coagulase(negative sta#hylococci. etiology. Follow u# MR: scanning with #hase( contrast seAuence is mandatory to veri%y #atency o% the stoma 40=(075.( 1.5. and e3tent o% neurologic damage #rior to correction o% the intracranial insult 4Figs 0 and -.ely to reAuire surgical treatment 40-5.. ventricular e3#ansion. &rologic #roblems because o% neuro#athic bladder usually reAuire intensive medical and surgical treatment 40. Krtho#edic #roblems. :t can be done %or #atients with H"P associated with myelomeningocele. such as scoliosis and %oot de%ormities. *reatment usually necessitates removal o% the shunt. also reAuire care%ul %ollow(u# because they are li. 'ndosco#ic third ventriculostomy has a success rate o% 7. )$4$ utcome *he overall outcome and #rognosis o% 9M is highly de#endent on various %actors including the age o% onset. Staphylococcus aureus has also been im#licated. :ntraventricular and intravenous antibiotics may be reAuired.5.

. with the main #roblems caused by the #hysical disabilities related to the level o% s#inal cord damage. :n the absence o% any com#le3 develo#mental syndrome.(7. Sim#le aAueduct stenosis is associated with very good outcome.5. At least =.. "hildren with myelomeningocele without H"P have normal intelligence. "hildren with s#ina bi%ida are e3#ected to have normal intellectual abilities. . *his is because o% the underlying brain #arenchyma damage 4-. Shunts have im#roved the outcome o% #atients with H"P dramatically.-0 40/5. Posthemorrhagic or #ostmeningitic H"P is associated with #oor outcome. #atients with H"P are e3#ected to survive and reach adulthood. which is considered normal 4005. and with care%ul treatment and %ollow(u#.F o% these #atients can attain an intelligence Auotient higher than /.

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--. Oarnes P et al. ?eurol "lin -00... Olennow M. 7-. Lil%ord R8 4eds5. 7. hitelaw A. -00=. C@ellmer :.. 66. *he #athogenesis o% neonatal #ost( hemorrhagic hydroce#halus. Love S.2 /: =+0(==-. 6/. 7. ?on(#rotein(bound iron is elevated in cerebros#inal %luid %rom #reterm in%ants with #osthemorrhagic ventricular dilatation. Olennow M. Practice #arameter: neuroimaging o% the neonate: re#ort o% the Huality Standards Subcommittee o% the American Academy o% ?eurology and the Practice "ommittee o% the "hild ?eurology Society. ?ilsson &A. 60. .. Savman C. :9H com#le3 o% lesions: cerebrovascular in@ury in the #reterm in%ant. Pediatr Res +. ?eurosurg "lin ? Am -00.=7(-.6.(. Par.. :n: Levene M:.ows. Arch $is "hild -0/=2 6. Canev PM. :ncidence and evolution o% sube#endymal and intraventricular hemorrhage: a study o% in%ants with birth weights less than -. "erebros#inal %luid #ressure during #ost haemorrhagic ventricular dilatation in newborn in%ants. Caiser AM. "herian S. ?eurosurgical management o% hydroce#halus. Allen ".ine res#onse in cerebros#inal %luid %rom #reterm in%ants with #osthaemorrhagic ventricular dilatation. Orain Pathol +. hitelaw A. Co%%ler H. *S.. &rlesberger O.+6 61.. *horesen M.=. SWvman C. 7+. Fetal and ?eonatal ?eurology and ?eurosurgery.=(. ?eurology +. Oada HS./.2 1: 6--(6-0.i '.: 0+. "hildRs ?erv Syst -00+2 /: .1. 6=.. gm. Pa#ile LA. 67. *he treatment o% hydroce#halus. 'dinburgh: "hurchill Livingstone. Ourstein 8. "yto. *horesen M.12 -1: ..=. Ment LR. ## 66-(666. hitelaw A. "orrelation o% ventricular siIe and head circum%erence a%ter severe intra(#eriventricular haemorrhage in #reterm in%ants..+2 0-: -. Hagberg H. Ourstein R. MTller $.+2 =/: -7+6(-7./(+-+.-2 10: +. Punt 8. 8 Pediatr -07/2 0+: =+0(=. *ar. Acta Paediatr +.(0+1. hitelaw A!.

7.. 'lbourne $. Pediatr ?eurosurg -00/2 +0: . $ra.erman "K. Philadel#hia: 70. ?eurosurgery -0062 . Pediatrics +.2 -.. "SF shunts =.. !ross S8./: 6. /1. Arch $is "hild. Cennedy "R. Ris. 8 ?eurosurg +.. 9entriculomegaly *rial !rou#. "am#bell M8..(-. Re#eated lumbar or ventricular #unctures in newborns with intraventricular haemorrhage. /. -00/. 8 Pediatr -0/. 8ohnson A.(/. '#stein ?'. years on((#ast. Hudgins R8. 7=..2 -6: /. Rosenthal A$. controlled trial o% acetaIolamide and %urosemide in #osthemorrhagic ventricular dilatation in in%ancy: %ollow(u# at . Li 9. Shunt #lacement and myelomeningocele .: .1. "hildRs ?erv Syst -0072 -./: =07(6. ?eurology o% the ?ewborn 41th ed5. 76.: 016(010. &se o% e3ternal drainage %or #osthemorrhagic hydroce#halus in very low birth weight #remature in%ants.- hitelaw A. %actors %or re#eated cerebros#inal shunt %ailures in #ediatric #atients with hydroce#halus. Ayers S. /-.. RandomiIed. O Saunders.0(.. Methods and com#lications in surgical cerebros#inal %luid shunting.7. igg M. $ra. "orni#s '. Plets ". *reatment o% #osthemorrhagic hydroce#halus in the #reterm in%ant with a ventricular access device. ?ormative early head growth in very(low(birth(weight in%ants../. Ksi#o%% M. Ho#e P. Cestle 8.2 0+: . /=. 7/. Ooydston R.60(.71. #resent and %uture. ?eurosurg "lin ? Am +..2 6=: . $ra. $evlieger H. $etermining the best cerebros#inal %luid shunt valve design: the #ediatric valve design trial.-. "ochrane Library :ssue .+7 71.e 8M. /+. "asaer P. -00.e 8M. 9ol#e 88.... Lawless 8. 'c.. *uli S.-(. 77. 9an "alenbergh F. "hildRs ?erv Syst +.. Cestle 8R. /.1(6.year. *uli S.-2 -. Uito 8. Randomised trial o% early ta##ing in neonatal #osthaemorrhagic ventricular dilatation.. Lamberti(Pasculli M.-2 -+: 6/=(60. !ilreath "L. +.e 8.

?eurosurg "lin ? Am +. Albright AL. /6. A review and recommendations. //. Pollac. 8ohnson $L.. $ias L.7.-2 -+: 7.1: -. 'arly re#air o% myelomeningocele and simultaneous insertion o% ventriculo#eritoneal shunt: techniAue and results. diagnosis. McLone $!.e 8M. Management o% hydroce#halus by endosco#ic third ventriculostomy in A. Myelomeningocele: a review o% the ortho#aedic as#ects o% +. M.. Pang $. $ev Med "hild ?eurol -00+2 . 0.. 07. /7.N+-+. ?eurosurgery . "hildRs ?erv Syst -0/=2 -: -1=(-17. Swan. 0-. treatment.. -0//. Stening -00. ?eurosurgery -0/72 +. PudenI RH. 0.6 #atients treated %rom birth with no selection criteria. '#idemiology.=: +. Oayston R. :rwin O8. Steinbo. Myelomeningocele re#air: technical considerations and com#lications.. *eo ".-.17(-. "are o% the neonate with a myelomeningocele. ?eurosurg "lin ? Am -00. ?eurosurg "lin ? Am +.-2 -+: 60=(7. Hydroce#halus: overdrainage by ventricular shunts. Shunt mal%unctions. Lo P. and #revention o% cerebros#inal %luid shunt in%ections. Ho%%man H8.. 8ones R. "ochrane $$. 'ndosco#ic third ventriculostomy. $ra. :F.: +-(+.+/ re#air: simultaneous vs seAuential shunting.. 0=. :rvine O. "om#arison o% simultaneous versus delayed ventriculo#eritoneal shunt insertion in children undergoing myelomeningocele re#air. Long(term outcome and com#lications o% children born with meningomyelocele. P. Review o% -+ cases./. $ra.(0... ?eurosurg "lin ? Am -00/2 0: ---(-+. 8ones RF. Miller P$. Orydon M. Pediatr ?eurosurg -0012 +-: /. "hildRs ?erv Syst -00+2 /: 0+(06.(.2 1: 6=7N666.e 8M: 9entriculostomy %or treatment o% hydroce#halus.=+. 0+. 06. Surg ?eurol -00-2 . /0. 8 "hild ?eurol -0062 --: .2 +6: /6N0+.(7. Hubballah MS. Mc"ullough $". 01. FoltI 'L.7+.

P. Long(term outcome and com#lications o% children born with meningomyelocele. -.: 7. $eeg CH. "hildRs ?erv Syst -00+2 /: 0+(06.+0 #atients with myelomeningocele.. Pulsed $o##ler sonogra#hic measurement o% normal values %or the %low velocities in the intracranial arteries o% healthy newborns. :ntelligence outcome in children with shunted hydroce#halus o% di%%erent etiology. Pantaleoni ". :rwin O8. Ho##e(Hirsch '. !iorgi ". "hildRs ?erv Syst -00/2 -1: 07(00. 0/.(7. . -. Pediatr Radiol -0/02 -0: 7-(7/. 00.-. UorIi ". Pediatr ?eurosurg -0062 +=: =7(6.. Laroussinie F. :rvine O. Riva $. Late outcome o% the surgical treatment o% hydroce#halus.. $evoti M. Orunet L et al.. "hildRs ?erv Syst -0012 -. Steinbo. "ochrane $$. Milani ?. Ru##recht *.

KbB!yn &ltrasound.er mal%ormation variant associated with cor#us callosal agenesis. 7.. Preterm neonate 4. Ooston5. =b.. Fig. Fig. Fig. Mild ventriculomegaly at -0 wee. Antenatal management o% %etal cerebral ventriculomegaly. wee. Follow(u# obstructive hydroce#halus at %oramen o% Monro at . /.s #ostnatal age5. KbB!yn &ltrasound.. ?eonatal management o% %etal ventriculomegaly. right %etal magnetic resonance imaging o% the same %etus 4Permission %rom Levine $ 4Associate Radiologist(in("hie% o% Academic A%%airs $irector. Fig.s %etus Permission %rom Levine $ 4Associate Radiologist(in("hie% o% Academic A%%airs $irector. Fetal ventriculomegaly Fig. Fig. Fig. Fig..+ wee. 1. . Figure Legends Fig. 6. Her brain magnetic resonance imaging revealed ventriculomegaly due to $andy( al. Fig. Ooston5. Oeth :srael $eaconess Medical "enter..month o% age. He was diagnosed as %etal . wee. 0. 9entriculomegaly associated with Arnold("hiari mal%ormation in a . Management o% neonatal hydroce#halus..s %etal li%e.s %etus. Fetal magnetic resonance imaging ventriculomegaly due to obstructive hydroce#halus at aAueduct o% Sylvius in a . at . Fig.wee. -. =a. wee. Oeth :srael $eaconess Medical "enter.s5 was diagnosed as neonatal hydroce#halus by ultrasonogra#hy at .1 wee. Later she has microce#haly with mild #sychomotor delay at the age o% + years. Fetal magnetic resonance imaging: 9entriculomegaly due to obstructive hydroce#halus at %oramen o% Monro in a +.s %etal li%e. ?eonatal ventriculomegaly associated with agenesis o% cor#us callosum 4acrocallosal syndrome: #olydactyl and agenesis o% cor#us callosum5.s %etus. -. Le%t: %etal ultrasonogra#hy with dilated lateral ventricle. +.

. .hydroce#halus but #roved venticulomegaly due to cerebral mal%ormation. He has moderate #sychomotor delay.