hydrocephalus in pineal and tectal tumors - link.springer.com · in contrast, tectal tumors also...

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Hydrocephalus in Pineal and Tectal Tumors Kenichi Nishiyama Contents Introduction ....................................................................................... 1 Pathophysiology of Hydrocephalus in Pineal and Tectal Tumors ............................... 2 Clinical Manifestations ........................................................................... 3 Radiological Findings ............................................................................ 4 Management ...................................................................................... 7 Outcome ........................................................................................... 13 References ........................................................................................ 14 Abstract Pineal and tectal tumors are most common brain tumors causing obstruction of the aqueduct and required CSF diversion procedures. Success rate of ETV in this type of hydrocephalus is denitely high. In addition, endoscopic approach allows for sampling of CSF, direct visualization of the tumor with surrounding structures, and biopsy for decision-making of therapeutic strategy. Therefore, simultaneous ETV and endoscopic biopsy has become an important procedure lately. MR images of sagittal section, especially with high resolution, are extremely useful for preoperative evaluation. Combination of trajectory and type of endoscope (i.e., rigid-, ber-, and videoscope) should be considered depending on changes in the ventricular anatomy as well as size of tumors and types of existing equipment. Keywords Pineal tumor · Tectal tumor · ETV · Aqueductoplasty · Dissemination K. Nishiyama (*) Department of Neurosurgery, Center for Neurological Diseases, Niigata Medical Center, Niigata, Japan e-mail: [email protected] # Springer Nature Switzerland AG 2018 G. Cinalli et al. (eds.), Pediatric Hydrocephalus, https://doi.org/10.1007/978-3-319-31889-9_56-1 1

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Page 1: Hydrocephalus in Pineal and Tectal Tumors - link.springer.com · In contrast, tectal tumors also cause obstruction of CSF pathway in the aqueduct and lead to a noncommunicating hydrocephalus;

Hydrocephalus in Pineal and Tectal Tumors

Kenichi Nishiyama

ContentsIntroduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1Pathophysiology of Hydrocephalus in Pineal and Tectal Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2Clinical Manifestations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3Radiological Findings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14

AbstractPineal and tectal tumors are most common brain tumors causing obstructionof the aqueduct and required CSF diversion procedures. Success rate of ETV inthis type of hydrocephalus is definitely high. In addition, endoscopic approachallows for sampling of CSF, direct visualization of the tumor with surroundingstructures, and biopsy for decision-making of therapeutic strategy. Therefore,simultaneous ETV and endoscopic biopsy has become an important procedurelately. MR images of sagittal section, especially with high resolution, areextremely useful for preoperative evaluation. Combination of trajectory andtype of endoscope (i.e., rigid-, fiber-, and videoscope) should be considereddepending on changes in the ventricular anatomy as well as size of tumors andtypes of existing equipment.

KeywordsPineal tumor · Tectal tumor · ETV · Aqueductoplasty · Dissemination

K. Nishiyama (*)Department of Neurosurgery, Center for Neurological Diseases, Niigata Medical Center,Niigata, Japane-mail: [email protected]

# Springer Nature Switzerland AG 2018G. Cinalli et al. (eds.), Pediatric Hydrocephalus,https://doi.org/10.1007/978-3-319-31889-9_56-1

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Introduction

Tumors of pineal and tectal plate are most common brain tumors causing obstructionof the aqueduct of Sylvius. These deep-seated lesions often present acute or subacuteobstructive hydrocephalus and necessitate CSF diversion procedures. Theoretically,endoscopic third ventriculostomy (ETV) creates more physiological cerebrospinalfluid (CSF) equilibrium and avoid many kinds of complications related to externalventricular drainage or placement of CSF shunt. According to the literatures, hydro-cephalus owing to pineal and tectal tumors is considered the best indication for ETV.Success rate of ETV in this hydrocephalus is definitely high (Dağlioğlu et al. 2003;Hellwig et al. 2005; Kulkarni et al. 2009; Sacko et al. 2010).

Resection of pineal and tectal tumors is not essential except for certain case;however, CSF sampling to evaluate the tumor markers or biopsy for histologicaldiagnosis is strongly required. Results of tumor markers or biopsy may contribute fordecision-making of therapeutic strategy of the tumors. Therefore, recently simulta-neous ETV coupled with endoscopic tumor biopsy has been recognized as favorableinitial diagnostic and therapeutic alternative (Constantini et al. 2013; Ellenbogen andMoores 1997; Morgenstern et al. 2011; Morgenstern and Souweidane 2013; O’Brienet al. 2006; Oi et al. 2000; Pople et al. 2001; Yamini et al. 2004). As neuroendoscopehas been a mainstream for procedure of choice for noncommunicating hydroceph-alus over the last two decades, endoscopic management in pediatric hydrocephaluswith these posterior third ventricular tumors will be applied as the focus in thischapter.

Pathophysiology of Hydrocephalus in Pineal and Tectal Tumors

Some authors consider the pathophysiology of hydrocephalus in posterior thirdventricular tumors as a more complicated mechanism.

Pineal region tumors typically manifest with noncommunicating hydrocephalusowing to obstruction of the entrance to the aqueduct early in the clinical course.Acute or subacute block of the aqueduct develops a pressure gradient between supra-and infratentorial spaces. It may lead to dilatation of the third ventricle. Block of CSFpathway at Sylvian aqueduct leads to disturbance in CSF moving between thirdventricle and peripheral subarachnoid space. Since it is regarded as one of the CSFabsorptive sites, increase in volume of bilateral and third ventricle may be graduallyinduced. On the other hand, capability of CSF absorption by the periventricularcapillaries is not enough to control CSF volume equilibrium in this pathologicalstate. The ventricular dilatation with increase in its pressure may compress thecortical veins. It leads to venous congestion (Greitz and Greitz 1997; Greitz 2004).In addition, deep venous drainage system is sometimes disturbed with “mass effect”by the deep-seated pineal region tumors. Therefore, the severe venous congestionwith brain swelling counteracted by ventricular dilatation develops more fatal statein acute phase of the hydrocephalus with pineal tumors (Fig. 1) (Cinalli et al. 2011).

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In contrast, tectal tumors also cause obstruction of CSF pathway in the aqueductand lead to a noncommunicating hydrocephalus; however, it is generally presentedas a chronic and compensated hydrocephalus. The intracranial compliance alreadydecreased following completed balance between the CSF production in the ventricleand the brain capillaries. Reduction of intracranial compliance may cause decrease inthe arterial expansion and increase in CSF pulsation. This CSF pulsation is consid-ered to have capability to induce transmantle pulsatile stress. It might be the force ofventricular dilatation even under normal CSF pressure (Cinalli et al. 2011; Di Roccoet al. 1978, 1979; Greitz 2004).

Clinical Manifestations

The pattern of symptoms due to pineal tumors depends on rate of growth andlocation. Generally, slow-growing tiny lesions remain asymptomatic until growingenough to cause obstruction of the entrance to the aqueduct. Symptoms secondary toobstructive hydrocephalus are usually the first to appear, followed by cranial nervedeficits including ocular movement. Some more benign tumors such as pineocytomamay be slow growing, causing any signs of obstructive hydrocephalus over time. Incontrast, fast-growing tumors such as pineoblastoma and germ cell tumors may haverapid onset and progression of symptoms. Acute symptoms of compression to thesurrounding structures or deterioration of mental status may occur due to secondaryintra-tumoral hemorrhage in some cases.

Pressure gradientbetween 3rd and 4th

ventricle

CSF moving limitation to subarachnoid space

Obstruction of the aqueduct

CSF volume in lateral and 3rd ventricle ↑

CSF absorption by the periventricular capillaries ↓

Venous congestion Disturbance in drainage of the deep venous system

Ventricular dilatation with increasing in pressure

Brain swelling

Located at pineal region

B

Fig. 1 Hypothesis of pathophysiological mechanism in hydrocephalus with pineal tumors

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The most common clinical symptom due to obstructive hydrocephalus at theaqueduct is intermittent headache increasing in severity and duration over time. It istypically worst after sleep. Sometimes nausea and vomiting are added. Patients maypresent in severe hydrocephalus and may develop altered mental status in moreadvanced stage. Pineal region tumors present various visual signs includingParinaud’s syndrome. Increased ICP induced by obstructive hydrocephalus cancause blurred vision, papilledema, and diplopia due to abducens palsy or compres-sion of the tectum.

Tectal tumors, usually tectal gliomas, are indolent remaining stable in size forseveral years (Stark et al. 2005; Ternier et al. 2006; May et al. 1991). Typically,symptoms are related to late-onset aqueductal stenosis, such as headache, nausea,and lethargy. Cranial nerve signs and long tract signs are uncommon findings despitethe location and intrinsic nature of the tumors. However, some tectal tumors mayfollow different growths, extending exophytically or infiltrating to the surroundingstructures (Dağlioğlu et al. 2003; Ternier et al. 2006). These tumors present cranialnerve signs including ocular movement disorders or ataxia.

Radiological Findings

Neuroradiological findings with usage of modern radiological techniques may helpfor correct diagnosis of the tumors, understanding about physiological condition, aswell as anatomical details in CSF pathway and selecting adequate treatment for bothtumors and hydrocephalus.

CT is still one of the standard examinations; however, its findings sometimes onlydemonstrate the presence of the tumor and imply the obstruction of the aqueductalorifice with suggestion by triventricular dilatation. CT may fail to reveal its presencein a case with typical small tectal tumor without any enhancement. In contrast, CT issuperior to MRI for visualization of the hemorrhage or calcification of the pinealtumors (Fig. 2). The incidence of calcification in children younger than 6 years isusually considered as abnormal. It should be evaluated for presence of the germ celltumors, such as germinomas, teratomas, and choriocarcinomas/NGGCTs.

MRI is essential for all cases of hydrocephalus in pineal and tectal tumors. MRIcan demonstrate anatomical abnormalities related to the noncommunicating hydro-cephalus with obstruction of the aqueduct. The most suitable images to evaluate theanatomical detail are a sagittal section. It shows clearly not only ventricular dilata-tion due to tumors that obstruct the aqueduct but also bulging of the third ventricularwall into the interpeduncular and chiasmatic cistern. The small intrinsic tectal tumorscan be visible (Fig. 3). In a case with pineal tumor, MRI is more useful fordetermining its origin, characterizing its extension, and revealing the presence ofdissemination (Fig. 4). High-resolution images such as 3D-CISS provide betterinformation for understanding details about ventricle and cistern, because of itsexcellent contrast between CSF and brain parenchyma. Additionally these high-resolution images are essential for preoperative evaluation of the endoscopic treat-ment. The presence of membranous structures causing additional obstruction of CSFpathway, tumor attachment to the ventricular wall, and heterogeneous component

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such as microcyst in the tumor can be revealed in a plain manner (Fig. 5) (Laitt et al.1999). By means of evaluating CSF physiological condition, sagittal view of T2WIis more effective. Flow void as a rapid CSF flow pulsation in the aqueduct is missingin almost all cases of noncommunicating hydrocephalus. Recently, MR imagingtechniques to visualize CSF movement such as phase-contrast (PC) MR imaging and

Fig. 3 Tectal glioma with obstructive hydrocephalus. (a) Sagittal T2-weighted image revealed asmall tectal mass (arrowhead) and dilatation of both lateral and third ventricles. (b) Sagittal sectionof CISS image clearly showing downward deviation of the third ventricular floor (arrow). A thickmembranous structure connected to the tectal tumor causes obstruction of the CSF pathway inmidportion of the aqueduct

Fig. 2 A 14-year-old boy,pineal germinoma withhydrocephalus. CT showingfine calcification on surface ofthe tumor (arrow)

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time-spatial labeling inversion pulse (Time-SLIP) have been frequently used. Thesemotion images provide effective information for understanding CSF movement inthe present progress form (Yamada et al. 2008, 2015).

Fig. 4 An 11-year-old boy, germinoma arising in suprasellar and pineal region. (a) Sagittal T1WIafter injection of gadolinium demonstrates tumors arising in both the suprasellar and the pinealregion. (b) Sagittal CISS image showing the irregular shaped tectal tumor occluding the aqueductcompletely. The floor of the third ventricle and the chiasm protrude outward

Fig. 5 A 14-year-old boy,immature teratoma arising inpineal region. A 3D-CISSimage showing the tumor witha large cyst and a solid partincludes microcysts

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Management

Advances in neuroendoscope have enhanced the intraventricular surgical tech-niques. In recent years, neuroendoscope has a major role to play in the managementof hydrocephalus owing to obstruction of CSF pathway by pineal and tectal tumors.It is true that CSF shunt has been the mainstay of treatment in these pathologicalconditions for a long time, but the problems associated with shunt insertion are wellknown. The advantages of ETV include avoidance of shunt-related complicationssuch as infection, overdrainage, and shunt dependency for a long time. The potentialfor obtaining biopsies from the tumor during the same procedure is also the strongpoint. Thus simultaneous endoscopic CSF diversion and biopsy has become animportant procedure lately (Miwa et al. 2015; Mohanty et al. 2011; O’Brien et al.2006; Oppido et al. 2011; Roth and Constantini 2015).

Hydrocephalus with pineal tumors is recognized as one of the best indications.Endoscopic approach in this region allows for direct visualization of the tumor,sampling of CSF for the tumor marker assay, and search for tumor dissemination(Al-Tamimi et al. 2008; Song et al. 2010). It enabled simultaneous treatment ofhydrocephalus by ETV and diagnosis for early management of the pineal tumors(Gangemi et al. 2001; O’Brien et al. 2006; Oi et al. 2000; Yamini et al. 2004). Toperform concurrent ETVand biopsy, trajectory and instrument should be consideredbefore the procedures (Fig. 6).

A single entry and trajectory is reasonable by meaning of less invasive forchildren. With usage of rigid endoscope, the compromised location of the burrhole setting between the ideal entry for ETV and biopsy has some disadvantages(Knaus et al. 2011; O’Brien et al. 2006; Oppido et al. 2011; Pople et al. 2001; Wonget al. 2011; Yurtseven et al. 2003) (Fig. 6a). A rigid endoscope may apply somepressure on the fornix during ETV and on the posterior border of the foramen ofMonro, which venous angle located nearby, during biopsy of the pineal tumors.Therefore, it should be indicated in a case with larger tumor located more anteriorthan the line of massa intermedia (Morgenstern and Souweidane 2013; Roth andConstantini 2015). Otherwise, two separate burr holes and trajectories should beplanned in a procedure with usage of rigid endoscope (Fig. 6b). A burr hole forETVis placed on less than 2 cm anterior from the coronal suture and on the mid-pupil line.Entry for endoscopic biopsy of the pineal tumors is considered to locate moreanterior part, sometimes on the hairline (Chibbaro et al. 2012; Veto et al. 1997;Yurtseven et al. 2003). Neuronavigation is useful and effective for determination ofthe entry sites. Some authors recommend routine use of navigation for the rigidendoscopic procedures (Kim et al. 2004; Roth and Constantini 2015).

Another technique to perform concurrent ETV and biopsy from a single entry isusing a flexible neuroendoscope (Fig. 6c) (Al-Tamimi et al. 2008; Ellenbogen andMoores 1997; Endo et al. 2009; Ferrer et al. 1997; Gangemi et al. 2001; Oppido et al.2011; Shono et al. 2007; Yamini et al. 2004). The strong point of the flexible scope isits capability of reaching both distant locations in the ventricle via single corticalpathway. It can easily reach any locations between the orifice to the aqueduct andposterior roof of the third ventricle, between anterior horn and trigonum of the lateralventricle, and even prepontine cistern via fenestration of the third ventricle after

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ETV. This characteristic is useful for observation to search disseminated tumors(Fig. 7). In addition, biopsy of a couple of different sites on the pineal tumor is easilypracticable with steerable scope (Fig. 8) (Roth and Constantini 2015). It regards astaking advantage, because germ cell tumors at pineal region occasionally displayheterogeneity and mixed cell populations within the same tumors.

Existing product of flexible scope was fiberscope. Fiberscope has a major limi-tation of resolution, because of capturing the images with glass fiber. In contrast,

Fig. 6 Three options for simultaneous ETV and endoscopic biopsy in hydrocephalus owing topineal tumors. (a) A single entry and trajectory with usage of rigid endoscope. The endoscope isinserted from a compromised entry point between ideal entry for ETV and biopsy. Pulling theendoscope in anterior and posterior direction is required to complete both ETVand biopsy. (b). Twoseparate entries and trajectories for rigid-endoscopic procedure. (c) A single entry and trajectorywith usage of flexible endoscope. The entry point is placed around the coronal suture for settingtrajectory parallel to the basilar artery (arrowhead: coronal suture line)

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current endoscope of flexible system is progressed to the videoscope. Videoscopehas a very small, miniature-sized CCD chip at the distal end of the scope. Placing theimaging device at the tip has made “on-site” processing of images without interrup-tion possible while maintaining consistent image quality (Fig. 9). The quality ofdigital images by videoscope is as high as those produced by rigid endoscope andeven operative microscope (Nishiyama et al. 2014; Oka 2008). It has a capability ofmagnifying observations of microscopic texture, such as capillary vessels, becauseof having a very short depth of field. Therefore, simultaneous ETV and biopsy withflexible videoscope has been recognized as a state-of-the-art surgery for hydroceph-alus due to pineal tumors. However, videoscope is not generally used to date. Usinga combined rigid-flexible endoscope to perform a single entry point is one of theoptions. The procedure is to use a rigid endoscope for inspection and evaluation ofthe tumor and performing an ETV and to use a flexible endoscope for completingbiopsy. It makes up for the disadvantage of unsatisfied visual quality by fiberscope(Depreitere et al. 2007; Oppido et al. 2011; Roth and Constantini 2015).

In a case with tectal tumors, ETV is considered as a first choice for treatment ofrelated hydrocephalus (Oka et al. 1999). However, endoscopic aqueductoplasty (EAP)has been described as another alternative for small mass lesions. EAP is theoreticallyconsidered to create a more physiological condition and provide long-term control.This procedure avoids the risk of severe arterial bleeding. However, the long-termresults of EAP have not been as successful as one would expect (Cinalli et al. 2006; daSilva et al. 2007; Ersahin 2007; Sagan et al. 2006; Schroeder et al. 2004). EAP hasbeen shown to fail frequently. In addition, EAP is generally considered as a riskier

Fig. 7 Endoscopic findings of disseminated germinoma in the right anterior horn. The fragiletumor is located adjacent to the right caudate head and anterior caudate vein. (a) Conventionalimage. (b) Narrowband image (NBI). Vasculatures in the tumor and subependymal veins are shownas brownish or cyan on NBI. NBI, an optical color separation filter narrows the bandwidth forspectral transmittance and lets only two narrow wavelengths through. These two specific wave-lengths are strongly absorbed by hemoglobin. As a result, the vascular structures were emphasizedon the display

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Fig. 8 Simultaneous ETV and endoscopic biopsy with videoscope for 11-year-old boy withhydrocephalus due to pineal germinoma (asterisk). ETV was performed with blunt perforationfollowed by balloon inflation (a). Subsequently, biopsy of three different sites of the tumor wascompleted (b). Endoscopic finding after the procedure demonstrates the ETV stoma (arrowhead)and accumulation of a small blood clot (arrow)

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procedure due to the higher risk of injuring midbrain structures. It may lead toneurological deficits such as oculomotor or trochlear nerve palsy, Parinaud’s syn-drome, and periaqueductal syndrome. Therefore, ETV, which has higher long-termsuccess rates and less risk, would be a better alternative for occlusion of the aqueduct(Fritsch and Schroeder 2013; Schroeder et al. 2000, 2012).

Tectal tumors are mostly benign astrocytic tumors, which are characterized asindolent and lack progression over time (May et al. 1991; Ternier et al. 2006). Smalltumors less than 4 cm3 are likely hamartoma (Dağlioğlu et al. 2003; Ternier et al.2006). The treatment is limited only to CSF diversion in principle and does notrequire removal or biopsy (Javadpour and Mallucci 2004; Stark et al. 2005; WellonsIII et al. 2002; Yeh et al. 2002). Although most tectal gliomas will never need furtherinterventions, a certain number will progress. It is rare, but successful ETV andconcomitant biopsy at the same time can be expected in these cases as same as pinealtumor cases (Fig. 10). Success rate of ETV both as initial procedure and after shuntfailure is definitely high for hydrocephalus due to tectal glioma (Dağlioğlu et al.2003; Javadpour and Mallucci 2004; Kulkarni et al. 2009; Li et al. 2005; Oka et al.1999; Romeo et al. 2013; Sacko et al. 2010; Wellons III et al. 2002). However, ETVfailure is a possible event. In the literature, repeat ETV also has a good success rate(Feng et al. 2004; Hellwig et al. 2013; Kadrian et al. 2005; Koch et al. 2002;Mohanty et al. 2002; Siomin et al. 2001; Surash et al. 2010). This is one optionfor patients with a failed ETV by means of avoiding to insert the shunt.

Fig. 9 A principle of the flexible videoscope. The videoscope has a mini-CCD chip at distal end ofthe scope. Placing the imaging device at the tip has made “on-site” processing without interruptionpossible while maintaining consistent image quality

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Which should be performed at first in the simultaneous operation, ETV orendoscopic biopsy? There is still controversy regarding the order. ETV is sometimesrecommended at first, as the deterioration is usually caused by hydrocephalus(Depreitere et al. 2007; Ferrer et al. 1997; Knaus et al. 2011; Oertel et al. 2009;Pople et al. 2001; Roth and Constantini 2015). The goal of endoscopic treatment isconsidered as improving any symptoms in principle. In contrast, some authorsrecommend that decrease in size of the third ventricle after ETV is unfavorable bymeans of maneuverability and safety in intraventricular endoscopic surgery.

Another point of controversy is a risk of dissemination (Chernov et al. 2006;Luther et al. 2010). According to many reports, the incidence rate of leptomeningealdissemination following endoscopic biopsy is considered very rare (Choi et al. 2007;Hayashi et al. 2011; Shono et al. 2007). However, it is undoubtedly a possiblecomplication. There are few case reports of tumor recurrence along the endoscopic

Fig. 10 Endoscopic procedure in hydrocephalus due to tectal glioma. (a) Tectal glioma withmoderate size (asterisk) extends into the aqueduct. The orifice of the aqueduct is demonstrated asa pinhole (arrow). (b) Biopsy of the tumor with forceps. Pineal recess (arrow) was revealed in anupward direction. (c) Perforating the paper-thin floor of the third ventricle with balloon

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tract in cases with pineal germ cell tumors, just like following stereotactic biopsy(Choi et al. 2007; Haw and Steinbok 2001; Rosenfeld et al. 1990; Talamonti et al.2013). The options to avoid dissemination are careful irrigation following biopsy,early induction of chemotherapy and radiotherapy, or extension of the radiation fieldin possible cases (Brown and Saran 2005; Haw and Steinbok 2001; Hayashi et al.2011; Talamonti et al. 2013).

A risk of hemorrhage is a point of attention in endoscopic biopsy. The techniquefor bleeding control is limited to only irrigation in a case with fragile tumors.Complicated massive intraventricular hemorrhage or intra-tumoral hemorrhagemay lead to more fatal state. The risk of subarachnoid hemorrhage may increase ina case with ETV followed by biopsy. Therefore, some authors emphasize that thesurgeons should choose an open biopsy or resection for the vascular-rich tumorsafter endoscopic CSF sampling and ETV.

Outcome

Kulkarni et al. reported a multicenter outcome analysis of ETV success for childrenwith hydrocephalus in a large cohort (n = 618). They found a 66% overall successrate, and among them 82% in patients with tectal tumors and 67% in patients with theother tumors. Sacko et al. also reported an analysis of 368 ETVs. They found asuccess rate of 85% in patients with tectal gliomas and 100% in pineal tumors.

Generally, factors indicating potential poor ETV outcome are considered veryyoung age, history of hemorrhage or infection, and shunt setting. To approximate thepredicted probability of ETV success, ETV success score (ETVSS) was validated in2009 (Fig. 11). With usage of ETVSS, one would expect 90% success rate of ETVasan initial treatment in a 10-year-old child with hydrocephalus due to tectal glioma(Kulkarni et al. 2009). Although success rate of ETV in early periods is high, ETV

ETV success score (ETVSS)= Age score + Etiology score + Previous shunt score

Score Age Etiology Previous Shunt

0 1mo post-infectious previous shunt

10 1mo to <6mos no-previous shunt

20myelomeningocele

IVHnontectal brain tumor

30 6mos to <1yraqueductal stenosis

tectal tumorothers

40 1yr to <10yrs

50 10 yrs

Fig. 11 ETV success score. (Modified from Kulkarni et al. 2009; Kim et al. 2004)

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failure occurs in some cases. According to the reports well known as “shunt designtrial” in 1998, the early failure rate of CSF shunt is similar to ETV. It is about30–40% (Drake et al. 1998). In contrast, the overall failure rate is about 59% for CSFshunt compared with 43% for ETV (Kestle et al. 2000; Kulkarni et al. 2009). Theauthors emphasize that the selective use of ETV would greatly reduce the number ofrepeat surgeries over the long term compared with CSF shunt.

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