ORIGINAL ARTICLE - ABSTRACT

Surgical treatment of long-standing overtventriculomegaly in adults (LOVA)

Ronak Ved1& Paul Leach1

& Chirag Patel1

Received: 28 July 2016 /Accepted: 13 October 2016 /Published online: 2 November 2016# The Author(s) 2016. This article is published with open access at Springerlink.com

AbstractBackground Longstanding overt ventriculomegaly in adults(LOVA) is characterised by chronic hydrocephalus presumedto begin during infancy, but arresting before becoming clini-cally detectable. Later in life clinical features of hydrocepha-lus ensue, typically in the 5th or 6th decades. Only a relativelysmall number of LOVA case series have been published, andambiguity remains regarding optimal management. This caseseries describes a series of patients with LOVA treated suc-cessfully at a single neurosurgical institution using endoscopicthird ventriculostomy (ETV).Methods A series of 14 patients were diagnosed with LOVAusing established clinical and radiological criteria. All patientsunderwent an ETVand their clinical conditions were followedup for up to 5 years post-operatively.Results Fourteen patients (100 %) reported either improve-ment or halt of progression in their presenting symptoms3 months after ETV; 93 % of patients (n = 13) did not requireany further surgical intervention. One patient (7 %) reporteddeterioration in symptoms beyond 3 months post-operatively,which necessitated further surgery (ventriculoperitonealshunt). These promising outcomes after ETV are mirrored innumerous other LOVA case series. Other works have analysedthe value of CSF shunting procedures in LOVA, with mixedresults. A direct, prospective comparison of outcomes aftershunt procedures and ETV, with a specific focus on LOVApatients, is yet to be completed. A minority of patients fail torespond, or develop recurrence of symptoms, months or yearsafter initial surgical intervention.

Conclusions ETVis an attractive option for surgical treatmentof LOVA. After surgical treatment for LOVA, long-term fol-low-up should be considered to screen for late recurrence ofthe condition.

Keywords Long-standing . Overt . Ventriculomegaly .

Adults . Hydrocephalus

Introduction

The term BLOVA^was first used by Oi and colleagues [1] todefine a cohort of adult patients with symptoms of chronichydrocephalus, a head circumference of more than 2 standarddeviations above the 98th percentile and overt tri-ventriculomegaly on neuroimaging, in the absence of a sec-ondary cause for aqueductal stenosis in adulthood. The mech-anism for this phenomenon remains unclear [2]. It ishypothesised that there is a full or partial obstruction of CSFflow through the aqueduct of Sylvius before fusion of cranialsutures (i.e. before age 24 months) followed by restoration ofCSF flow before clinical symptoms canmanifest in childhood.This theory explains the large head circumference and initialasymptomatic period of patients with true LOVA. This resto-ration in CSF may be explained by re-establishment of flowthrough the aqueduct, use of alternative flow pathways, mod-ification of CSF production or a combination of these mech-anisms [1, 3].

Later in adulthood, ill-defined alterations in CSF flow dy-namics lead to a return of the previously arrested hydroceph-alus and thus the gradual onset of symptoms of hydrocepha-lus, such as headaches, cognitive decline4, imbalance, visualproblems and psychological disturbance [4]. Neuroimagingtypically reveals triventricular hydrocephalus with sparing ofthe fourth ventricle and absence of aqueductal stenosis.

* Ronak Vedvedr@cf.ac.uk

1 University Hospital of Wales, Cardiff CF14 4XW, UK

Acta Neurochir (2017) 159:71–79DOI 10.1007/s00701-016-2998-7

Exemplar T2-weighted magnetic resonance images of a pa-tient with LOVA are given in Fig. 1.

The process is typically slow and progressive in nature,and a loss in brain parenchyma plasticity makes treatmentoutcomes of LOVA difficult to predict. There are conflict-ing case series describing outcomes for patients withLOVA after neuroendoscopic and shunt-based CSF diver-sion procedures, with each series advocating differentopinions as to the optimal management for this debilitatingcondition.

This case series collates outcome data from LOVA patientstreated using endoscopic third ventriculostomy (ETV) at ourinstitution and reviews current data published about the pro-posed pathophysiology, diagnosis, treatment options and pa-tient outcomes for LOVA.

Methods

Fourteen patients were diagnosed with LOVA at a single neu-rosurgical unit between 2011 and 2016, by virtue of all the

patients meeting the clinical and radiological criteria forLOVA established by Oi et al. [2] (Table 1). All patientsunderwent an ETV utilising the same standardised operativetechnique, outlined by Al-Jumaily et al. [4]. A single burr holewas performed in the midpupillary line just anterior to thecoronal suture. Disposable rigid endoscopes were used to cre-ate a stoma in the floor of the third ventricle using a figure-of-eight balloon. If present, any secondary membrane in the pre-pontine cistern was opened.

Outcomes were assessed by clinical review of patients inoutpatient clinics (minimum 6 months, maximum 5 years)post-operatively. If discharged from clinic, patients and theirprimary care physicians were asked to contact the neurosurgi-cal unit if symptoms recurred [6].

MEDLINE® database searches were conducted to retrievearticles related to LOVA. Search terms included Blong-stand-ing overt ventrioculomegaly in adults^, BLOVA^, Badulthydrocephalus^, Badult ventriculomegaly^ and Barrestedhydrocephalus^. Two reviewers independently scrutinisedeach retrieved article, evaluating the relevance of the study’ssources and analysed the main findings of each study. A

Fig. 1 T2-weighted magneticresonance images from a patientwith LOVA: Case 14 in our caseseries. a and b Sagittal slicesdemonstratingtriventriculomegaly, with sparingof the cerebral aqueduct andfourth ventricle (thin whitearrows). There is apparentenlargement of the sella turcicadue to the chronic nature of thehydrocephalus (thick whitearrow). c Coronal slicedemonstrating obliteration of theseptum pellucidum (black arrow).The triventriculomegaly can onceagain be identified. d Subtlesulcal effacement may beappreciated, particularly in theright temporo-parietal region, onthis axial slice from the samescan. However, this patient’s age-related (81) cerebral atrophyrenders this effacement lessmarked than in typical cases ofLOVA (where presentation is inthe 5th–6th decades)

72 Acta Neurochir (2017) 159:71–79

summary of the results from the studies deemed relevant foranalysis is outlined in Table 2.

Results

Table 3 depicts the demographics, presenting symptoms andoutcomes for the 14 cases of LOVA treated at our institutionfrom 2011 to 2016. There were eight males and six females,with a mean age of 51 (22–81). All 14 patients met the diag-nostic criteria outlined in Table 1. All patients underwent ETVin the first instance. Follow-up ranged from 6 to 60 months(median 36 months). The mean head circumference was58.2 cm.

One patient (7 %) experienced a post-operative complica-tion after ETV in the form of a single tonic-clonic seizureimmediately post-operatively; there were no other complica-tions post-ETV. All 14 patients (100 %) reported either im-provement or halt of progression in their presenting symptoms3 months after ETV; 93 % of patients (n = 13) did not requireany further surgical intervention. Only one patient (7 %) re-ported deterioration in symptoms beyond 3 months that ne-cessitated further surgery (VP shunt). Two patients have re-cently reported minor headaches at 4 and 5 years follow-uppost-operatively and are awaiting review from the seniorauthor.

Discussion

Demographics

The clinical onset of LOVA can occur at any stage in adult-hood, with a range between 22 and 81 years (median 54) inour case series, which has been mirrored by others [4, 7, 8].No clear-cut differences in patient sex have been identifiedthus far. Some series have identified a trend towards sub-normal IQ and cognitive abilities in LOVA patients, althoughthis feature does not appear to be universal across all cases of

LOVA [4, 7, 8]. Given the progressive nature of LOVA, earlyidentification of patients with potential symptoms and signs ofthe condition is an attractive notion, as it may help to maxi-mise the impact of any surgical intervention to implement itearlier in the disease process. However, the subtle myriad ofearly symptoms and the non-specific nature of large head cir-cumference make early detection of LOVA a clinicalchallenge.

Pathophysiology

The key features of LOVA are aqueductal stenosis, with arrestof hydrocephalus before the onset of gross macrocephaly andthe symptoms of raised ICP. During the arrested hydrocephal-ic period CSF flow is likely maintained via a combination ofrencannalisation of the aqueduct, utilisation of alternative CSFflow routes, and modification of CSF production or absorp-tion [1]. This asymptomatic interval is followed later in life bya failure of the compensatory processes, leading to the symp-tomatic phase of LOVA, which is typically progressive andlong term. Patients with an identifiable insult to alter CSF floware excluded from the definition of true LOVA, and the mech-anisms that alter CSF dynamics to end the asymptomaticphase of the condition remain unclear.

It is thought that the symptoms of LOVA develop as aconsequence of (1) failure of adequate CSF flow, (2) skullbase changes as a consequence of chronic and progressive risein ICP and (3) chronic pressure effects upon brain parenchyma[1, 9, 10]. As such, early intervention has the potential tomodify the disease process before irreversible changes occuras a result of chronically raised ICP [4].

Reports of persistent symptoms in patients with LOVAafter both ETVand shunt procedures have led to a theory that,in some cases, the pathological process in adulthood leadingto CSF volume imbalance may actually take place more dis-tally than the aqueduct; it may be a phenomenon of failure ofCSF re-absorption, thus unnameable to traditional CSF diver-sion procedures. One case reported byRekate [13] illustrated aLOVA patient whose symptoms remained refractory to CSFshunt diversion and ETV, which in itself was complicated byshunt infection. A venogram revealed bilateral transverse si-nus stenosis, which was successfully treated utilising a neuro-radiological venous-stenting procedure. The patient’s ICPsubsequently normalised. It is possible that back-pressurefrom blockade of dural venous sinuses could instigate a chron-ic, potentially reversible, aqueductal stenosis.

Symptomatology

Al-Jumaily et al. identified the most common features inLOVA were headaches and imbalance [4], which were alsothe most common presenting symptoms in our case series(Table 3).

Table 1 Clinical and radiological criteria used to confirm the diagnosisof LOVA in the presented case series (adapted from Oi et al., 2000 [1])

1. Clinical symptoms of hydrocephalus developing in adulthood—e.g.headaches, cognitive decline, imbalance, gait disturbance,psychological disturbance, visual deterioration/diplopia

2. Macrocephaly defined by head circumference >98th percentile inadulthood (male 53.8 cm; female 52.9 cm) [5, 14]

3. Overt tri-ventriculomegaly (lateral and third ventricles) onneuroimaging, with cortical sulcal effacement and/or destruction ofthe sella turcica as evidence of long-standing ventriculomegaly

4. Absence of a secondary cause for aqueductal stenosis in adulthood(e.g. previous meningitis, subarachnoid haemorrhage)

Acta Neurochir (2017) 159:71–79 73

Tab

le2

Summaryof

MEDLIN

E®

results

forpublishedcase

series

ofLOVApatientstreatedwith

CSF

diversion,viashunto

rETV

Authors(year)

No.of

LOVA

patients

(totalin

cohort)

Prim

aryinterventio

ns(n)

Outcomes

Com

plications

Caveats

Oietal.(2000)

[1]

18(18)

ETV(8);VPshunt(9)

(DPV

7;PP

V2)

Clin

icalim

provem

entand

radiologicalarrestof

hydrocephalusin

all

patientsafterprim

ary

ETVandPPV

SDHin

all7

patientsreceivingDPV

shunts,

requiringshuntrevisionto

PPV

orETV

Twointra-axialh

aemorrhages

afterETV.

One

case

oftransientv

isualfield

deficit

afterETV

Relativelysm

allcohort

Keiferetal.(2002)

23(23)

Gravitatio

nal-shunt(23)

82%

(19)

patientsreporting

symptom

aticim

provem

ent

2SDH—onenecessitatin

goperative

drainage

Non-com

parativ

estudy;

potentialselectio

nbias;relativelysm

allcohort

Keiferetal.(Jan2005)

30(30)

Gravitatio

nalshunt

(30)

87%

(26)

patientsreported

improvem

entin

pre-operativesymptom

s

2post-operativ

ehygrom

as—onerequiring

shuntrevision;

limitedor

transient

improvem

entinpre-operativesymptom

sin

3(10%)patients

Non-com

parativ

estudy;

follo

w-upof

upto

12monthsonly,therefore

lateshunt

infections

orfailu

respotentially

not

captured;p

otentialselectio

nbias;

relativ

elysm

allcohort

Keiferetal.(July

2005)

26(26)

Gravitatio

nalshunt

(26)

87%

(22)

ofpatients

reportingclinical

improvem

ent

2SDH;4

caseswith

symptom

sof

over-drainage,2requiringshuntrevision;

onecase

ofsevere

weightg

ain(due

tounderdrainage)

requiringreplacem

ento

ftheshuntAssistant®

portionof

theshunt;

onecase

with

recurrence

ofsymptom

s

Non-com

parativ

estudy;

patient

selection

potentially

affected

bysuboptim

alETV

equipm

ent;relativ

elysm

allcohort

Canuetal.(2005)[7]

1(1)

N/A

Identificationof

preserved

language

andpraxis

functio

nsdespite

severe

ventriculomegaly

N/A

Singlecase

report;n

otherapeutic

interventio

nsperformed

RekateH.(2007)[13]

6(6)

ETV(6)

CSF

flow

throughETV

confirmed

radiologically

inallcases

post-operativ

ely

Persistent

symptom

sin

5cases(83%)

necessitatingVPshunto

rvenous

stentin

g.Mild

short-term

mem

orydeficits

in1patient

Smallcohort;heterogeneouspatient

populatio

n,includingthree(50%)

patients<30

andone<20

(age

16).ETVs

with

inthestudycohortperformed

attwo

differentinstitutions

Ham

anda

etal.(2009)[8]

1(1)

ETV(1)

Improvem

entinheadaches,

mem

ory,cognitive

and

constructio

nalabilities

None

Singlecase

report;p

atient

hadahistoryof

operated

myeloschisisandaqueductal

stenosisas

achild

(potentially

nota

true

case

ofLOVA)

Jenkinsonetal.(2009)[6]

24(190)

ETV(24)

88%

(21)

reportingim

proved

symptom

safterETV

9post-ETVcomplications

inthetotalcohort

(5%):2minor

SDHs;2transientfocal

neurologicaldeficits;2

CSFleaks;3ICHs

(2necessitatin

gEVDplacem

ent)

Heterogeneity

ofindicatio

nsforETV;small

numbersof

patientsin

each

subgroup;

outcom

esdefinedby

clinicalassessment

inoutpatient

clinic

AlJum

aily

etal.(2012)[4]

20(20)

ETV(20)

Improvem

entinheadache

(18;

90%);im

proved

balance(12;

80%)

Persistent

headachesin

2patients(10%),

requiringrepeatETVandgravitatio

nal

shuntinsertio

n,with

persistenceof

symptom

sdespite

shuntinsertio

nin

one

patient.T

woshortseizuresim

mediately

post-operativ

ely;

poor

cognitive

Patientsfailing

torespondto

ETVmay

have

been

sufferingfrom

non-ICP-related

chronicdaily

headaches—

however

ICP

monito

ring

was

notp

erform

edto

confirm

this

74 Acta Neurochir (2017) 159:71–79

The same group also highlighted that patients with LOVAmay demonstrate a myriad of cognitive and psychologicalproblems such as decline in memory, attention and languageskills, along with depression, anxiety and disinhibition [4, 7,8]. Some case series have retrospectively identified that a pro-portion of LOVA patients demonstrated sub-normal IQs orevidence of early dementia before the onset of more overtsymptoms of raised ICP, such as headaches and gait distur-bance [4, 7, 8]. However other patients demonstrate normal-to-high IQs and report no evidence of hydrocephalic symp-toms at childhood, with a large head circumference the onlypre-symptomatic indicator of a potential CSF flow problem[3, 4]. Whether any pre-symptomatic decline in cognitivefunction identified in a proportion of LOVA patients is a con-sequence of the arrested hydrocephalus at childhood or wheth-er it represents the earliest phase of the decompensation ofCSF flow in adulthood remains to be explored.

These symptoms may be difficult to identify clinically, asthe chronic nature of LOVA may permit some patients’ brainsundergo sufficient neuroplasticity to permit functionalreorganisation, minimising any clinical neuropsychologicaldeficits until late in the disease process [4, 8]. Nevertheless,when cognitive decline, inattention and mood disturbances dooccur in LOVA, they can have significant impacts upon qualityof life [4]. These symptoms were marked in one case from thecohort at our institution. In this case there was significant im-provement in the social functioning, memory and mood afterETV (Table 3). Whilst there are other reports of improvementin these faculties after surgical treatment of LOVA [8], it isthought that these symptoms are less likely to improve if treat-ment is implemented late in the disease process [4].Neuropsychological symptoms should therefore be assessedand considered early in the therapeutic decision-making pro-cess for LOVA patients who may appear to function highlyeven after the point of clinical decompensation ensues [4, 6–8].

Treatment

All reviewed studies advocated surgery for symptomatic casesof LOVA. However, the optimal CSF diversion procedure forpatients with LOVA has been debated for over a decade [1, 2,9–12]. This debate has centred on a debate betweenneuroendoscopic procedures versus CSF shunt diversion. Oiet al. [1] reported outcomes for 18 patients with LOVA, 9undergoing neuroendoscopic ETV (8) or aqueduct-plasty (1)and 9 receiving ventriculoperitoneal (VP) shunts [7 differen-tial pressure valve (DPV) shunts, 2 pressure programmablevalve (PPV) shunts]. All seven patients receiving DPV shuntsdeveloped subdural haematomas, most likely as a conse-quence of over-drainage. All of these patients required shuntrevision to a PPV shunt and/or ETV. Six of the nine patientsundergoing neuroendoscopic procedures developed radiolog-ical and clinical arrest of hydrocephalus. Two patients in thisT

able2

(contin

ued)

Authors(year)

No.of

LOVA

patients

(totalin

cohort)

Prim

aryinterventio

ns(n)

Outcomes

Com

plications

Caveats

performancespersistedacross

thecohort

post-ETV

Ono

etal.(2012)[10]

1(1)

Pressure

programmable

valveVPshunt(1)

Improvem

entinmem

ory

SDHrequiringdrainage

andsecond

shunt

insertion

Singlecase

report

Issacs

etal.(2016)

97(163)

ETV(163)

130(87%)of

totalp

atient

cohortreported

improvem

entinsymptom

sat3monthspost-ETV

10(6

%):meningitis

(4);SDH(2);focal

neurologicaldeficit(1);m

emorydeficit

(1);weightg

ain(2).Nolong-term

disability

Datanotspecificto

LOVA(cohortincludes

ETVsforfailedVPshunts,secondary

hydrocephalusandNPH

)

Ved

etal.(2016)

14(14)

ETV(14)

14patients(100

%)reported

improvem

ento

rhaltof

progressionin

presentin

gsymptom

s3months

post-operativ

ely;

97%

(13)

One

(7%)post-operativ

eseizurewith

nolong-term

disability;

one(7

%)patient

requiringasecond

procedure(V

Pshunt)

dueto

symptom

recurrence

Relativelysm

allcohort.Outcomes

defined

byclinicalassessmentinoutpatient

clinic

SDH=subduralhaem

atom

a;NPH

=norm

alpressure

hydrocephalus;ICH=intracerebralh

aemorrhage

Acta Neurochir (2017) 159:71–79 75

Tab

le3

Caseseries

of14

patientsdiagnosedwith

LOVAandtreatedwith

prim

aryETV.O

utcomes

werereview

edat3monthsandsubsequently

between6months–5yearspost-operativ

ely

Case

number

Sex

Presentingsymptom

sAge

atpresentatio

nHead

circum

ference

Operativ

ecomplications

Outcomeat3months

Outcomebeyond

3months

1M

Leg

weakness,falls,H

eadaches

4355

cmNo

Reduced

headache

frequency,B80%

better^

5years:no

deterioration;

B100

%better^

2M

Unsteadiness

3858

cmNo

Nodeteriorationin

balance

5years:no

deterioration

3M

Dizzy

spells,m

oodsw

ings

and

headaches

5362

cmNo

Improved

mem

oryandheadaches;no

deteriorationin

moodsw

ings

5years:mild

headachesanddizzinessreturned

4F

Poor

mobility

andheadaches

6859

cmNo

Improved

mobility

andheadachesabsent

5years:im

proved

mobility

andheadaches

5M

Ataxia,seizures,and

poor

mem

ory

3454

cmYes

(Generalised

tonic

clonicseizure)

Improved

ataxiaandseizurefrequency;

nodeteriorationof

mem

ory

5years:no

furtherdeterioration

6F

Poor

mobility

andheadaches

5759

cmNo

Improved

mobility

andheadaches

4years:return

ofheadaches;aw

aitin

goutpatient

review

7M

Unsteadiness

6360

cmNo

Balance

improved

3years:Bbalance

back

tonorm

al^

8M

Diplopia

5960

cmNo

Diplopiaresolved

36months:return

ofdiplopiaandonseto

flegweakness:

VPshuntinserted

9F

Imbalance

7557

cmNo

Improved

balanceandmobility

2years:no

furtherdeterioration

10M

Headaches

andim

balance

2258

cmNo

Improved

headachesandbalance

2years:balancefurtherim

proved

11M

Headaches

andcognitive

decline

5559

cmNo

Improved

headachesandcognition

1year:cognitio

nfurtherim

proved

12F

Headaches

4159

cmNo

Improved

headaches

1year:n

ofurtherdeterioration

13F

Headaches

1757

cmNo

Headaches

absent

1year:n

ofurtherdeterioration

14F

Mem

orydisturbance,disinhibition,

andunsteadiness

8158

cmNo

Socially

andcognitively

muchim

proved

at4months

6months:continuedcognitive

andsocialim

provem

ent

76 Acta Neurochir (2017) 159:71–79

study suffered intra-axial haemorrhages after ETV. However,subsequent series have demonstrated the relative safety ofETV, with an overall complication risk of approximately6 % [4, 13]. In one case series, a single ETV provided long-term symptomatic relief for 88 % (21/24) of patients withLOVA with no long-term complications port-operatively [6].This correlates with our series, where there was only onecomplication post ETV: a self-limiting generalised seizurewith no long-term sequelae for the patient. Conversely100% (n = 14) of patients in our cohort reported improvementor absence of progression in their symptoms at their firstfollow-up appointment after ETV (3 months).

There are no case reports of significant change in ventric-ular diameter after ETV or shunt insertion. The explanationtouted for this is that only a small volume of CSF needs to bedrained to achieve near-physiological ICP in adult hydroceph-alus [11]. Improvements in radiological CSF flow and ICPafter ETV for LOVA have been identified [1, 13]. However,these do not appear to correlate with clinical improvement,and recurrence of symptoms does not reliably relate to failureof flow through the ETV stoma [13]. It seems that after initialdrainage of CSF through the stoma, a more complicatedreorganisation of CSF dynamics takes place. Unpacking thismechanism may then be the key to understanding how toachieve sustained relief from the symptoms of LOVA.

The majority of studies, our series included, demonstratethat a majority of LOVA patients can achieve post-operativeclinical improvement in headaches, balance, motor skills andneuropsychological function after ETV [3, 4, 7, 8]. One largeretrospective study of ETV in adult patients (n = 163) recentlyidentified that the majority of patients undergoing primaryETV (87 %; n = 130) reported subjective improvement insymptoms 3 months post-operatively [3]. Only ten patients(6 %) suffered post-operative complications. It should be not-ed that the authors present data relating to their cohort as awhole, which encompassed adult patients with normal pres-sure hydrocephalus, partially treated childhood hydrocephalus(i.e. failed VP shunt), hydrocephalus secondary to other intra-cranial pathologies and LOVA. Separate outcome data forthese specific causes of adult hydrocephalus were not present-ed; however, 97 patients in the whole cohort (60 %) did meetthe diagnostic criteria for LOVA, and their outcomes for ETVsuccess and complications align with data from other caseseries specifically reviewing cases of LOVA (Table 2).

Despite these impressive outcomes after ETV, other worksreport less promising results. In one series, six patients withLOVA underwent ETV [10]. All six patients required a second-ary procedure for persistent symptoms: shunt insertion, repeatETVor venous stenting. This small series highlighted the needfor continued follow-up whatever intervention is implemented,as 50 % of patients (n = 3) suffered recurrence of symptoms at18–36 months after their primary ETV, despite an initial im-provement or arrest in progression of symptoms. Other studies

subsequently identified varying long-term success rates forETVs carried out for secondary hydrocephalus and LOVA,with recurrence of symptoms occurring a number of monthsor years post-operatively in a small proportion of patients insome series [1, 4, 7–10]. Furthermore, studies have presentedpatients in whom ETV did not facilitate objective improvementin cognitive or neuropsychological tests, which may reflect theprogressive nature of the condition and inter-individual differ-ences in neural compliance after a period of chronic hydroceph-alus [4]. In patients with recurrent symptoms repeat ETV orshunt insertion can sometimes deliver symptomatic improve-ment, but this is again unpredictable. Thus, decisions to re-operate need to be guided by individual patient circumstances.Many cases of LOVA treated with ETV, including those in ourseries, have demonstrated a benefit in the short-to-mediumterm. However long-term follow-up, over a period of years,should be considered, given the reports of recurrence and pro-gression of symptoms in a minority of LOVA patients up to 2–3 years post-operatively [4, 10, 13].

Two major advantages of ETV over traditional shuntingprocedures are the high infection (and thus revision) ratesfor shunt devices and the risk of shunt over-drainage and itssequale in the latter. After the dramatic complications follow-ing PPV insertion in the initial series by Oi et al., focus shiftedonto use of gravitational shunts for LOVA patients to mini-mise the risk of over-drainage [1, 2, 11, 12]. In an importantstudy of shunt procedures for LOVA patients, 23 patientselected gravitational shunt insertion after being offered thechoice of ETV or shunt insertion [11, 12]. Two patients(9 %) developed small subdural haematomas, one of whichrequired drainage. Eighty-two percent (19) of the cohort dem-onstrated clinical improvement after shunt insertion (follow-up 6–75 months). The authors conclude that gravitationalshunts are a viable alternative to ETV in the management ofLOVA. However, it is of note that 15 % (4) of patients devel-oped symptoms of overdrainge post-operatively, two of whichnecessitated shunt revision surgery. Median follow-up was29 months, which may not have been extensive enough tocapture cases of later shunt failure or infection in the cohort.Furthermore, the work was limited by the non-randomisedmethodology for the selection of patients for shunt insertionand the institution’s relatively large, rigid endoscopes leadingto strict criteria for excluding ETV as a therapeutic option(foramen of Munro width <6.5 mm and third ventricle floorthickness >2.5 mm).

When discussing treatment options for LOVA, the signifi-cant infection and revision rates associated with shunt proce-dures, and the risks of under- or over-drainage, must be con-sidered [4]. Conversely, the ventricular anatomy may not al-ways be amenable to endoscopic CSF diversion procedures. Acomparative study between ETV and shunt implantation forLOVA is yet to be completed; such work could provide usefuldata to aid therapeutic decision-making for these patients.

Acta Neurochir (2017) 159:71–79 77

Conclusion

The mysterious pathophysiology underlying LOVAmakes therapeutic decision-making for these patientscomplicated. Unchecked, its progressive nature can leadto morbidity, with poor cognitive, psychological andneurological outcomes [4].

It is vital that patients are counselled about the fact thatsurgical intervention for LOVA does not bring with it a guar-antee of long-term symptomatic relief or neuropsychologicalimprovement. The compliance of brain parenchyma is highlyvariable between individuals and the capacity for recoveryafter intervention does not appear to correlate with radiologi-cal arrest of hydrocephalus [1, 11, 13].

However, the potential for slowing or halting progres-sion via an established and safe procedure such as ETVmakes it an attractive therapeutic option for both clinicianand patient. ETVobviates many of the the risks associatedwith CSF diversion implant procedures, but gravitationalshunts may be considered in cases unsuitable for ETV orafter recurrence of symptoms after initial ETV [2, 11, 12].Such recurrence may occur months to years after initialETV. Long-term follow-up should be implemented aftersurgery for LOVA.

Compliance with ethical standards

Funding No funding was received for this research.

Conflict of interest All authors certify that they have no affiliationswith or involvement in any organization or entity with any financialinterest (such as honoraria; educational grants; participation in speakers’bureaus; membership, employment, consultancies, stock ownership, orother equity interest; and expert testimony or patent-licensing arrange-ments), or non-financial interest (such as personal or professional rela-tionships, affiliations, knowledge or beliefs) in the subject matter or ma-terials discussed in this manuscript.

The authors declare that they have no conflicts of interest.

Ethical approval All procedures performed in studies involving hu-man participants were in accordance with the ethical standards of theinstitutional and/or national research committee and with the 1964Helsinki Declaration and its later amendments or comparable ethicalstandards.

Informed consent Informed consent was obtained from all individualparticipants included in the study.

Open Access This article is distributed under the terms of the CreativeCommons At t r ibut ion 4 .0 In te rna t ional License (h t tp : / /creativecommons.org/licenses/by/4.0/), which permits unrestricted use,distribution, and reproduction in any medium, provided you give appro-priate credit to the original author(s) and the source, provide a link to theCreative Commons license, and indicate if changes were made.

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Comments

Any new and serious article on the LOVA condition is worthwhile as itdeals with a largely underestimated clinical problem. Therefore the ini-tiative to edit a review on the respective literature as well as presentingown experiences is valid. Only a few comments in order to intensify thediscussion should be allowed: it is important to broaden the definition theLOVA condition in the sense that aqueductal stenosis or typical three-ventricular hydrocephalus is a common, but not the only finding in thesepatients. Many ventriculomegalic patients with cystic CSF spaces of theposterior fossa (Blake’s pouch, Dandy-Walker variant, megacisterna

78 Acta Neurochir (2017) 159:71–79

magna, etc.) are found to develop typical LOVA symptoms, sometimesalready in early adulthood. Although some of the patients may havelimited relief from their symptoms after treatment, the long-term progno-sis is frustrating for both patient and neurosurgeon and very much com-parable to typical idiopathic NPH.

Therefore it must be strongly recommended to prevent this conditionby early detection and treatment of so-called asymptomatic or arrestedhydrocephalus during childhood.

Angela-Martina Messing-JungerBonn, Germany

Acta Neurochir (2017) 159:71–79 79